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Marvisi C, Macaluso F, Ricordi C, Cavazza A, Muratore F, Salvarani C. Diagnostic approach in giant cell arteritis. Autoimmun Rev 2025; 24:103743. [PMID: 39793744 DOI: 10.1016/j.autrev.2025.103743] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2024] [Revised: 01/06/2025] [Accepted: 01/07/2025] [Indexed: 01/13/2025]
Abstract
Giant cell arteritis (GCA), also known as temporal arteritis, is the most common form of vasculitis in the elderly. While initially described as involving the temporal arteries, GCA can also affect the aorta and its major branches. Despite the increased use of imaging modalities and the availability of temporal artery biopsy, diagnosing GCA remains challenging. GCA should be considered a spectrum, with diagnostic methodologies tailored to the prevalent symptoms. The sensitivity and specificity of different diagnostic approaches can vary depending on the clinical setting. Timing in diagnosing GCA is crucial to prevent serious complications, such as blindness and cerebrovascular ischemic events. While the prompt initiation of glucocorticoids (GCs) has reduced the incidence of major ischemic events, an uncertain diagnosis may expose the patient to unnecessary harm, such as complications from overtreatment or organ damage due to inadequate control of vasculitis. This narrative review will summarize the most widely available diagnostic techniques for GCA and outline our approach for cases where the diagnosis may be uncertain.
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Affiliation(s)
- Chiara Marvisi
- Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy; University of Modena and Reggio Emilia, Modena, Italy
| | - Federica Macaluso
- Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - Caterina Ricordi
- Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy; University of Modena and Reggio Emilia, Modena, Italy
| | - Alberto Cavazza
- Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - Francesco Muratore
- Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy; University of Modena and Reggio Emilia, Modena, Italy
| | - Carlo Salvarani
- Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy; University of Modena and Reggio Emilia, Modena, Italy.
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Hamann S, Ing EB, Lee AG, Van Stavern GP. Can Ultrasound Replace Temporal Artery Biopsy for Diagnosing Giant Cell Arteritis? J Neuroophthalmol 2024; 44:273-279. [PMID: 38551663 DOI: 10.1097/wno.0000000000002132] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/15/2024]
Affiliation(s)
- Steffen Hamann
- Department of Ophthalmology (SH), Rigshospitalet, University of Copenhagen, Denmark; Department of Ophthalmology & Visual Sciences (EI), University of Alberta, Edmonton, Canada; Chair of Ophthalmology (AGL), Blanton Eye Institute, Methodist Hospital, Houston, Texas; and Department of Ophthalmology and Visual Sciences (GPVS), Washington University in St. Louis, St. Louis, Missouri
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Pouncey AL, Yeldham G, Magan T, Lucenteforte E, Jaffer U, Virgili G. Halo sign on temporal artery ultrasound versus temporal artery biopsy for giant cell arteritis. Cochrane Database Syst Rev 2024; 2:CD013199. [PMID: 38323659 PMCID: PMC10848297 DOI: 10.1002/14651858.cd013199.pub2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2024]
Abstract
BACKGROUND Giant cell arteritis (GCA) is a systemic, inflammatory vasculitis primarily affecting people over the age of 50 years. GCA is treated as a medical emergency due to the potential for sudden, irreversible visual loss. Temporal artery biopsy (TAB) is one of the five criteria of the American College of Rheumatology (ACR) 1990 classification, which is used to aid the diagnosis of GCA. TAB is an invasive test, and it can be slow to obtain a result due to delays in performing the procedure and the time taken for histopathologic assessment. Temporal artery ultrasonography (US) has been demonstrated to show findings in people with GCA such as the halo sign (a hypoechoic circumferential wall thickening due to oedema), stenosis or occlusion that can help to confirm a diagnosis more swiftly and less invasively, but requiring more subjective interpretation. This review will help to determine the role of these investigations in clinical practice. OBJECTIVES To evaluate the sensitivity and specificity of the halo sign on temporal artery US, using the ACR 1990 classification as a reference standard, to investigate whether US could be used as triage for TAB. To compare the accuracy of US with TAB in the subset of paired studies that have obtained both tests on the same patients, to investigate whether it could replace TAB as one of the criteria in the ACR 1990 classification. SEARCH METHODS We used standard Cochrane search methods for diagnostic accuracy. The date of the search was 13 September 2022. SELECTION CRITERIA We included all participants with clinically suspected GCA who were investigated for the presence of the halo sign on temporal artery US, using the ACR 1990 criteria as a reference standard. We included studies with participants with a prior diagnosis of polymyalgia rheumatica. We excluded studies if participants had had two or more weeks of steroid treatment prior to the investigations. We also included any comparative test accuracy studies of the halo sign on temporal artery US versus TAB, with use of the 1990 ACR diagnostic criteria as a reference standard. Although we have chosen to use this classification for the purpose of the meta-analysis, we accept that it incorporates unavoidable incorporation bias, as TAB is itself one of the five criteria. This increases the specificity of TAB, making it difficult to compare with US. We excluded case-control studies, as they overestimate accuracy, as well as case series in which all participants had a prior diagnosis of GCA, as they can only address sensitivity and not specificity. DATA COLLECTION AND ANALYSIS Two review authors independently assessed the studies for inclusion in the review. They extracted data using a standardised data collection form and employed the QUADAS-2 tool to assess methodological quality. As not enough studies reported data at our prespecified halo threshold of 0.3 mm, we fitted hierarchical summary receiver operating characteristic (ROC) models to estimate US sensitivity and also to compare US with TAB. We graded the certainty of the evidence using the GRADE approach. MAIN RESULTS Temporal artery ultrasound was investigated in 15 studies (617 participants with GCA out of 1479, 41.7%), with sample sizes ranging from 20 to 381 participants (median 69). There was wide variation in sensitivity with a median value of 0.78 (interquartile range (IQR) 0.45 to 0.83; range 0.03 to 1.00), while specificity was fair to good in most studies with a median value of 0.91 (IQR 0.78 to 1.00; range 0.40 to 1.00) and four studies with a specificity of 1.00. The hierarchical summary receiver operating characteristic (HSROC) estimate of sensitivity (95% confidence interval (CI)) at the high specificity of 0.95 was 0.51 (0.21 to 0.81), and 0.84 (0.58 to 0.95) at 0.80 specificity. We considered the evidence on sensitivity and specificity as of very low certainty due to risk of bias (-1), imprecision (-1), and inconsistency (-1). Only four studies reported data at a halo cut-off > 0.3 mm, finding the following sensitivities and specificities (95% CI): 0.80 (0.56 to 0.94) and 0.94 (0.81 to 0.99) in 55 participants; 0.10 (0.00 to 0.45) and 1.00 (0.84 to 1.00) in 31 participants; 0.73 (0.54 to 0.88) and 1.00 (0.93 to 1.00) in 82 participants; 0.83 (0.63 to 0.95) and 0.72 (0.64 to 0.79) in 182 participants. Data on a direct comparison of temporal artery US with biopsy were obtained from 11 studies (808 participants; 460 with GCA, 56.9%). The sensitivity of US ranged between 0.03 and 1.00 with a median of 0.75, while that of TAB ranged between 0.33 and 0.92 with a median of 0.73. The specificity was 1.00 in four studies for US and in seven for TAB. At high specificity (0.95), the sensitivity of US and TAB were 0.50 (95% CI 0.24 to 0.76) versus 0.80 (95% CI 0.57 to 0.93), respectively, and at low specificity (0.80) they were 0.73 (95% CI 0.49 to 0.88) versus 0.92 (95% CI 0.69 to 0.98). We considered the comparative evidence on the sensitivity of US versus TAB to be of very low certainty because specificity was overestimated for TAB since it is one of the criteria used in the reference standard (-1), together with downgrade due to risk of bias (-1), imprecision (-1), and inconsistency (-1) for both sensitivity and specificity. AUTHORS' CONCLUSIONS There is limited published evidence on the accuracy of temporal artery US for detecting GCA. Ultrasound seems to be moderately sensitive when the specificity is good, but data were heterogeneous across studies and either did not use the same halo thickness threshold or did not report it. We can draw no conclusions from accuracy studies on whether US can replace TAB for diagnosing GCA given the very low certainty of the evidence. Future research could consider using the 2016 revision of the ACR criteria as a reference standard, which will limit incorporation bias of TAB into the reference standard.
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Affiliation(s)
| | - Geoffrey Yeldham
- Department of Ophthalmology, Cardiff & Vale University Health Board, Cardiff, UK
| | - Tejal Magan
- Kings College NHS Foundation Trust, London, UK
| | - Ersilia Lucenteforte
- Department of Statistics, Computer Science, Applications "G. Parenti", University of Florence, Florence, Italy
| | - Usman Jaffer
- Imperial College Healthcare NHS Trust, London, UK
| | - Gianni Virgili
- Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
- IRCCS- Fondazione Bietti, Rome, Italy
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Kaandorp BI, Raterman HG, Stam F, Gamala M, Meijer‐Jorna LB, Kalb FB, Wallis JW. Determination of the Value of Color Doppler Ultrasound in Patients With a Clinical Suspicion of Giant Cell Arteritis. ACR Open Rheumatol 2024; 6:56-63. [PMID: 37997540 PMCID: PMC10867289 DOI: 10.1002/acr2.11628] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2023] [Revised: 09/20/2023] [Accepted: 10/09/2023] [Indexed: 11/25/2023] Open
Abstract
OBJECTIVE It is urgent to diagnose giant cell arteritis (GCA) as quickly as possible to prevent irreversible blindness. The traditional gold standard for diagnosing GCA is temporal artery biopsy (TAB). However, TAB lacks diagnostic performance and carries out risks of surgical intervention. The noninvasive color Doppler ultrasound (CDU) seems to be a promising alternative. This study is designed to assess the diagnostic value of CDU in daily clinical practice. METHODS In this prospective cohort study, patients with a clinical suspicion of active GCA were included and underwent a CDU of the temporal arteries. If deemed necessary by the referrer, a TAB and/or 18F-fluorodeoxyglucose positron emission tomography with computed tomography was performed. The retrospective clinical diagnosis was determined 1 year after inclusion by two physicians experienced in the field of vasculitis. RESULTS 242 patients were included and GCA was diagnosed in 73 (30%) patients by the defined retrospective clinical diagnosis. Compared with the retrospective diagnosis, CDU has a sensitivity of 60% (48-72), specificity of 94% (89-97), positive predictive value (PPV) of 81% (70-89), negative predictive value (NPV) of 85% (80-88), and an accuracy of 84% (78-88). A total of 84 (35%) patients underwent TAB. TAB has a sensitivity of 66% (51-79), specificity of 100% (90-100), PPV of 100% (100), NPV of 67% (58-75), and an accuracy of 80% (70-88). CONCLUSION This study shows comparable diagnostic performance for CDU and TAB and even better CDU results with a bilateral halo present. Considering the advantages of the noninvasive CDU, it is the diagnostic tool of choice.
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van Nieuwland M, Colin EM, Boumans D, Vermeer M, Brouwer E, Alves C. Diagnostic delay in patients with giant cell arteritis: results of a fast-track clinic. Clin Rheumatol 2024; 43:349-355. [PMID: 37650990 PMCID: PMC10774201 DOI: 10.1007/s10067-023-06739-w] [Citation(s) in RCA: 4] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2023] [Revised: 08/01/2023] [Accepted: 08/08/2023] [Indexed: 09/01/2023]
Abstract
Giant cell arteritis (GCA) can lead to severe complications if left untreated. The aim of this study was to describe time from onset of symptoms to diagnosis and treatment in GCA suspected patients in a fast-track clinic (FTC), and secondarily to assess the influence of GCA symptoms on this time. A retrospective cohort consisting of suspected GCA patients who visited the FTC between January 2017 and October 2019 was used. Time between symptom onset, first general practitioner visit, FTC referral, first FTC visit, and treatment initiation was analysed. Furthermore, this was stratified for subtypes of GCA and GCA symptoms. Of 205 patients referred with suspected GCA, 61 patients received a final diagnosis of GCA (GCA+) and 144 patients had no GCA (GCA-). Median time after onset of symptoms to first FTC visit was 31.0 days (IQR 13.0-108.8) in all referred patients. Time between onset of symptoms and first GP visit was 10.5 (4.0-36.3) days, and time between first GP visit and FTC referral was 10.0 (1.0-47.5) days. Patients were generally seen at the FTC within 1 day after referral. For patients with isolated cranial GCA (n = 41), median delay from onset of symptoms to treatment initiation was 21.0 days (11.0-73.5), while this was 57.0 days (33.0-105.0) in patients with extracranial large-vessel involvement (n = 20) (p = 0.02). Our results indicate considerable delay between symptom onset and FTC referral in patients suspected of GCA. Suspected patients were examined and GCA+ patients were treated instantly after referral. Key Points • GCA can cause severe complications with delayed treatment, but non-specific symptoms make diagnosis challenging. • Diagnostic delay still occurs despite introducing a successful fast-track clinic resulting from delay between start of symptoms and FTC referral. • Patients who presented with constitutional symptoms had longer delay than patients who presented with isolated cranial symptoms.
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Affiliation(s)
- Marieke van Nieuwland
- Department of Rheumatology and Clinical Immunology, Ziekenhuisgroep Twente (Hospital Group Twente), Almelo, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
| | - Edgar M Colin
- Department of Rheumatology and Clinical Immunology, Ziekenhuisgroep Twente (Hospital Group Twente), Almelo, The Netherlands
| | - Dennis Boumans
- Department of Rheumatology and Clinical Immunology, Ziekenhuisgroep Twente (Hospital Group Twente), Almelo, The Netherlands
| | - Marloes Vermeer
- ZGT Academy, Ziekenhuisgroep Twente (Hospital Group Twente), Almelo, The Netherlands
| | - Elisabeth Brouwer
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
| | - Celina Alves
- Department of Rheumatology and Clinical Immunology, Ziekenhuisgroep Twente (Hospital Group Twente), Almelo, The Netherlands
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Moreel L, Betrains A, Doumen M, Molenberghs G, Vanderschueren S, Blockmans D. Diagnostic yield of combined cranial and large vessel PET/CT, ultrasound and MRI in giant cell arteritis: A systematic review and meta-analysis. Autoimmun Rev 2023; 22:103355. [PMID: 37146926 DOI: 10.1016/j.autrev.2023.103355] [Citation(s) in RCA: 19] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2023] [Accepted: 04/30/2023] [Indexed: 05/07/2023]
Abstract
OBJECTIVES To estimate the diagnostic accuracy of combined cranial and large vessel imaging by PET/CT, ultrasound and MRI for giant cell arteritis (GCA). METHODS PubMed, Embase, Cochrane and Web of Science databases were searched from inception till August, 312,022. Studies were included if they involved patients with suspected GCA and assessed the diagnostic accuracy of combined cranial and large vessel imaging by PET/CT, ultrasound or MRI with the final clinical diagnosis as reference standard. RESULTS Eleven (1578 patients), 3 (149 patients) and 0 studies were included for the diagnostic accuracy of ultrasound, PET/CT and MRI, respectively. Combined cranial and large vessel ultrasound had a sensitivity of 86% (76-92%) and specificity of 96% (92-98%). PET/CT of both cranial and large vessels yielded a sensitivity of 82% (61-93%) and specificity of 79% (60-90%). No studies assessed both PET/CT and ultrasound, which precluded head-to-head comparison. Addition of large vessel ultrasound to ultrasound of the temporal arteries (7 studies) significantly increased sensitivity (91% versus 80%, p < 0.001) without decrease in specificity (96% versus 95%, p = 0.57). Evaluating cranial arteries in addition to large vessels on PET/CT (3 studies) tended to increase the sensitivity (82% versus 68%, p = 0.07) without decrease in specificity (81% versus 79%, p = 0.70). CONCLUSION Combined cranial and large vessel ultrasound and PET/CT provided excellent accuracy for the diagnosis of GCA. Either PET/CT or ultrasound may be preferred depending on setting, expertise and clinical presentation. The diagnostic accuracy of combined cranial and large vessel MRI needs to be determined in future studies.
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Affiliation(s)
- Lien Moreel
- Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium; Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium.
| | - Albrecht Betrains
- Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium; Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium
| | - Michaël Doumen
- Department of Rheumatology, University Hospitals Leuven, Leuven, Belgium; Department of Development and Regeneration, Skeletal Biology and Engineering Research Centre, KU Leuven, Leuven, Belgium
| | - Geert Molenberghs
- Interuniversity Institute for Biostatistics and Statistical Bioinformatics (I-BioStat), University of Leuven and Hasselt University, Leuven, Belgium
| | - Steven Vanderschueren
- Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium; Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium; European Reference Network for Immunodeficiency, Autoinflammatory, Autoimmune and Pediatric Rheumatic disease (ERN-RITA)
| | - Daniel Blockmans
- Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium; Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium; European Reference Network for Immunodeficiency, Autoinflammatory, Autoimmune and Pediatric Rheumatic disease (ERN-RITA)
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Diagnostic validity of ultrasound including extra-cranial arteries in giant cell arteritis. Clin Rheumatol 2023; 42:1163-1169. [PMID: 36357632 DOI: 10.1007/s10067-022-06420-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2022] [Revised: 09/26/2022] [Accepted: 10/19/2022] [Indexed: 11/12/2022]
Abstract
OBJECTIVES Color Doppler ultrasound (CDUS) of the temporal arteries (TA) is becoming the first test to be performed for suspected giant cell arteritis (GCA). Our aim was to assess the added value of including CDUS of large vessels (LV) in the diagnosis of GCA. METHODS We performed an observational and retrospective study of consecutive patients with suspected GCA. Baseline CDUS of the TA and LV (axillary, subclavian, and carotid) were conducted. We defined the CDUS finding as positive if the halo sign was present. RESULTS Of 198 patients with suspected GCA, 87 were eventually diagnosed with GCA: 45 (51.7%) had a cranial pattern exclusively, 31 (35.6%) had both a cranial and an LV pattern, and 11 (12.6%) had an isolated LV pattern. CDUS of the TA had a sensitivity of 83.9%, specificity of 97.3%, and positive and negative predictive values (PPV, NPV) of 96.1% and 88.5%, respectively. When LV was added, sensitivity increased to 96.6% and NPV to 98.2%. Specificity was 97.3% and PPV was 96.6%. As for LVs, the axillary, subclavian, and carotid arteries were involved in 87.8%, 77.4%, and 34.4%, respectively. Isolated axillary examination resulted in a loss of 12.2% of patients with LV involvement; however, inclusion of the axillary and subclavian arteries retained 100% of patients with LV involvement. CONCLUSIONS Detection of GCA by ultrasound should routinely include examinations of the TA and LV (at least the axillary and subclavian arteries) to improve diagnostic accuracy. More than 12% of patients in our cohort had isolated LV involvement. Key Points • Extracranial involvement in GCA is very common: half of patients have extracranial vasculitis and more than 12% isolated LV involvement that can be demonstrated with CDUS. • Adding a CDUS examination of LV to TA increased sensitivity (from 83.9 to 96.6%) and the negative predictive value (from 88.5 to 98.2%) for diagnosis of GCA. • In our cohort, if we only examined the axillary arteries, 12.2% of the CGA with LV involvement would not have been diagnosed. • We propose a CDUS protocol that includes examination of the TA and LV (at least the axillary and subclavian arteries) routinely in cases of suspected GCA.
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Nakajima E, Moon FH, Junior NC, Macedo CR, de Souza AWS, Iared W. Accuracy of Doppler ultrasound in the diagnosis of giant cell arteritis: a systematic review and meta-analysis. Adv Rheumatol 2023; 63:5. [PMID: 36755336 DOI: 10.1186/s42358-023-00286-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2022] [Accepted: 01/29/2023] [Indexed: 02/10/2023] Open
Abstract
BACKGROUND Giant cell arteritis (GCA) is the most common primary systemic vasculitis in people 50 years of age and over, and it is considered a medical emergency due to the potential risk of permanent visual loss. Color Doppler ultrasound (CDU) of the temporal arteries is a rapid, noninvasive method to diagnose GCA. This study aims to determine the diagnostic accuracy of the halo sign in temporal arteries by CDU in people with suspected GCA. METHODS The systematic literature review included the search for publications in the following electronic databases: PubMed, Embase, CENTRAL, LILACS, WHO ICTRP, ClinicalTrials.gov, gray literature up to December 2022, and no date or language restrictions were applied. We analyzed studies including patients over 50 years of age with suspected GCA evaluating CDU of temporal arteries as a diagnostic tool against clinical diagnosis as a standard reference. Paper titles and abstracts were selected by two investigators independently for all available records. The quality of the studies was assessed using the Quality of Diagnostic Accuracy Studies tool (QUADAS-2) and the R software (version 4.2.1) was used for data analysis. The protocol of this review is registered with PROSPERO (CRD42016033079). RESULTS Twenty-two studies including 2893 participants with suspected GCA who underwent temporal artery CDU were evaluated. The primary analysis results showed a sensitivity of 0.76 [95% confidence interval (95 CI) 0.69-0.81] and specificity of 0.93 (95 CI 0.89-0.95) when the halo sign was compared to clinical diagnosis. The sensitivity value of 0.84 (95 CI 0.72-0.92) and specificity of 0.95 (95 CI 0.88-0.98) were found in five studies involving 1037 participants that analyzed the halo sign and temporal artery compression sign. A sensitivity of 0.86 (95 CI 0.78-0.91) and specificity of 0.95 (95 CI 0.89-0.98) were found in four studies with 603 participants where the halo sign was evaluated CDU on temporal and axillary arteries. CONCLUSION The detection of the halo sign by CDU of temporal arteries has good accuracy for the diagnosis of cranial GCA. The compression sign in temporal arteries and the addition of axillary arteries assessment improves the diagnostic performance of CDU for GCA. TRIAL REGISTRATION PROSPERO CRD42016046860.
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Affiliation(s)
- Eliza Nakajima
- Department of Evidence-Based Health, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Napoleão de Barros, 865, São Paulo, 04024-002, Brazil.,Division of Vascular and Endovascular Surgery, Department of Surgery, Escola Paulista de Medicina - Universidade Federal de Sao Paulo, Rua Borges Lagoa, 754, Sao Paulo, 04038-002, Brazil
| | - Francisca Hatta Moon
- Division of Vascular and Endovascular Surgery, Department of Surgery, Escola Paulista de Medicina - Universidade Federal de Sao Paulo, Rua Borges Lagoa, 754, Sao Paulo, 04038-002, Brazil
| | - Nelson Carvas Junior
- Department of Evidence-Based Health, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Napoleão de Barros, 865, São Paulo, 04024-002, Brazil
| | - Cristiane Rufino Macedo
- Department of Evidence-Based Health, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Napoleão de Barros, 865, São Paulo, 04024-002, Brazil
| | - Alexandre Wagner Silva de Souza
- Rheumatology Division, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua dos Otonis, 863, São Paulo, SP, 04025-002, Brazil.
| | - Wagner Iared
- Department of Evidence-Based Health, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Napoleão de Barros, 865, São Paulo, 04024-002, Brazil
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Monjo-Henry I, Fernández-Fernández E, Mostaza JM, Lahoz C, Molina-Collada J, de Miguel E. Ultrasound halo count in the differential diagnosis of atherosclerosis and large vessel giant cell arteritis. Arthritis Res Ther 2023; 25:23. [PMID: 36788547 PMCID: PMC9926809 DOI: 10.1186/s13075-023-03002-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2022] [Accepted: 01/30/2023] [Indexed: 02/16/2023] Open
Abstract
OBJECTIVE To determine the diagnostic discriminant validity between large vessel giant cell arteritis (LV-GCA) and atherosclerosis using ultrasound (US) intima-media thickness (IMT) measurements. METHODS We included 44 patients with LV-GCA and 42 with high-risk atherosclerosis. US examinations of the axillary, subclavian, and common carotid arteries (CCA) were systematically performed using a MylabX8 system (Genoa, Italy) with a 4-15-MHz probe. IMT ≥ 1 mm was accepted as pathological. RESULTS The LV-GCA cohort included 24 females and 20 males with a mean age of 72.8 ± 7.6 years. The atherosclerosis group included 25 males and 17 females with a mean age of 70.8 ± 6.5 years. The mean IMT values of all arteries included were significantly higher in LV-GCA than in atherosclerosis. Among LV-GCA patients, IMT ≥ 1 mm was seen in 31 axillary, 30 subclavian, and 28 CCA. In the atherosclerotic cohort, 17 (38.6%) had IMT ≥ 1 mm with axillary involvement in 2 patients, subclavian in 3 patients, carotid distal in 14 patients (5 bilateral), and isolated carotid proximal affectation in 1 case. A cutoff point greater than 1 pathological vessel in the summative count of axillary and subclavian arteries or at least 3 vessels in the count of six vessels, including CCA, showed a precision upper 95% for GCA diagnosis. CONCLUSION The IMT is higher in LV-GCA than in atherosclerosis. The proposed US halo count achieves an accuracy of > 95% for the differential diagnosis between LV-GCA and atherosclerosis. The axillary and subclavian arteries have higher discriminatory power, while carotid involvement is less specific in the differential diagnosis.
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Affiliation(s)
- Irene Monjo-Henry
- Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain.
| | - Elisa Fernández-Fernández
- grid.81821.320000 0000 8970 9163Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain
| | - José María Mostaza
- grid.81821.320000 0000 8970 9163Department of Internal Medicine, Hospital Carlos III, Madrid, Spain
| | - Carlos Lahoz
- grid.81821.320000 0000 8970 9163Department of Internal Medicine, Hospital Carlos III, Madrid, Spain
| | - Juan Molina-Collada
- grid.410526.40000 0001 0277 7938Rheumatology Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Eugenio de Miguel
- grid.81821.320000 0000 8970 9163Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain
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Noumegni SR, Jousse-Joulin S, Hoffmann C, Cornec D, Devauchelle-Pensec V, Saraux A, Bressollette L. Comparison of halo and compression signs assessed by a high frequency ultrasound probe for the diagnosis of Giant Cell Arteritis. J Ultrasound 2022; 25:837-845. [PMID: 35426608 PMCID: PMC9705675 DOI: 10.1007/s40477-021-00618-3] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2021] [Accepted: 08/18/2021] [Indexed: 10/18/2022] Open
Abstract
OBJECTIVE To evaluate the diagnosis performances of halo and compression signs alone and combined, assessed by a high frequency 22-MHz probe, and test their agreement in giant cell arteritis (GCA). METHODS In this cross-sectional study on patients suspected with GCA, halo sign was defined as hypo or iso-echogenic circumferential aspect of the vessel wall in transverse or longitudinal view; and compression sign was defined as visibility of the vessel wall upon transducer-imposed compression of the artery. Agreement of the two signs was tested using the Cohen's kappa statistic. RESULTS A total of 80 patients (50% women) were included with a mean age of 74.4 years. Twenty participants (25%) were ultimately treated for GCA. Halo and compression signs have respective prevalences of 35% and 48%, with respective sensitivity and specificity of 80% and 80% for the halo sign; and 85% and 65% for the compression sign. The kappa coefficient for the global agreement of the two signs was 0.67 (95% confident interval: 0.54-0.85). Combination of the two signs give a sensitivity of 80% and a specificity of 81.7%. CONCLUSION Halo and compression signs assessed by a high frequency probe, show a good level of agreement for the diagnosis of GCA and improve ultrasound specificity when combined together.
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Affiliation(s)
- Steve Raoul Noumegni
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, EA 3878 GETBO, Brest, France.
- EA3878 (GETBO), Brest University, Brest, France.
| | - Sandrine Jousse-Joulin
- Rheumatology Department, Brest Teaching Hospital, Brest University, INSERM, LBAI, UMR1227 Brest, Brest, France
| | - Clément Hoffmann
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, EA 3878 GETBO, Brest, France
- EA3878 (GETBO), Brest University, Brest, France
| | - Divi Cornec
- Rheumatology Department, Brest Teaching Hospital, Brest University, INSERM, LBAI, UMR1227 Brest, Brest, France
| | - Valérie Devauchelle-Pensec
- Rheumatology Department, Brest Teaching Hospital, Brest University, INSERM, LBAI, UMR1227 Brest, Brest, France
| | - Alain Saraux
- Rheumatology Department, Brest Teaching Hospital, Brest University, INSERM, LBAI, UMR1227 Brest, Brest, France
| | - Luc Bressollette
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, EA 3878 GETBO, Brest, France
- EA3878 (GETBO), Brest University, Brest, France
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11
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Kirby C, Flood R, Mullan R, Murphy G, Kane D. Evolution of ultrasound in giant cell arteritis. Front Med (Lausanne) 2022; 9:981659. [PMID: 36262280 PMCID: PMC9574015 DOI: 10.3389/fmed.2022.981659] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2022] [Accepted: 08/26/2022] [Indexed: 11/13/2022] Open
Abstract
Ultrasound (US) is being increasingly used to diagnose Giant Cell Arteritis (GCA). The traditional diagnostic Gold Standard has been temporal artery biopsy (TAB), but this is expensive, invasive, has a false-negative rate as high as 60% and has little impact on clinical decision-making. A non-compressible halo with a thickened intima-media complex (IMC) is the sonographic hallmark of GCA. The superficial temporal arteries (STA) and axillary arteries (AA) are the most consistently inflamed arteries sonographically and imaging protocols for evaluating suspected GCA should include at least these two arterial territories. Studies evaluating temporal artery ultrasound (TAUS) have varied considerably in size and methodology with results showing wide discrepancies in sensitivity (9–100%), specificity (66–100%), positive predictive value (36–100%) and negative predictive value (33–100%). Bilateral halos increase sensitivity as does the incorporation of pre-test probability, while prior corticosteroid use decreases sensitivity. Quantifying sonographic vasculitis using Halo Counts and Halo Scores can predict disease extent/severity, risk of specific complications and likelihood of treatment response. Regression of the Halo sign has been observed from as little as 2 days to as late as 7 months after initiation of immunosuppressive treatment and occurs at different rates in STAs than AAs. US is more sensitive than TAB and has comparable sensitivity to MRI and PET/CT. It is time-efficient, cost-effective and allows for the implementation of fast-track GCA clinics which substantially mitigate the risk of irreversible blindness. Algorithms incorporating combinations of imaging modalities can achieve a 100% sensitivity and specificity for a diagnosis of GCA. US should be a standard first line investigation in routine clinical care of patients with suspected GCA with TAB reserved only for those having had a normal US in the context of a high pre-test probability.
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Affiliation(s)
- Colm Kirby
- Department of Rheumatology, Tallaght University Hospital and Trinity College Dublin, Dublin, Ireland,Department of Rheumatology, Cork University Hospital and University College Cork, Cork, Ireland,*Correspondence: Colm Kirby
| | - Rachael Flood
- Department of Rheumatology, Tallaght University Hospital and Trinity College Dublin, Dublin, Ireland
| | - Ronan Mullan
- Department of Rheumatology, Tallaght University Hospital and Trinity College Dublin, Dublin, Ireland
| | - Grainne Murphy
- Department of Rheumatology, Cork University Hospital and University College Cork, Cork, Ireland
| | - David Kane
- Department of Rheumatology, Tallaght University Hospital and Trinity College Dublin, Dublin, Ireland
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12
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López-Gloria K, Castrejón I, Nieto-González JC, Rodríguez-Merlos P, Serrano-Benavente B, González CM, Monteagudo Sáez I, González T, Álvaro-Gracia JM, Molina-Collada J. Ultrasound intima media thickness cut-off values for cranial and extracranial arteries in patients with suspected giant cell arteritis. Front Med (Lausanne) 2022; 9:981804. [PMID: 36091695 PMCID: PMC9459085 DOI: 10.3389/fmed.2022.981804] [Citation(s) in RCA: 18] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2022] [Accepted: 08/11/2022] [Indexed: 12/01/2022] Open
Abstract
Objective To determine the optimal ultrasound (US) cut-off values for cranial and extracranial arteries intima media thickness (IMT) to discriminate between patients with and without giant cell arteritis (GCA). Methods Retrospective observational study including patients referred to an US fast-track clinic. All patients underwent bilateral US examination of the cranial and extracranial arteries including the IMT measurement. Clinical confirmation of GCA after 6 months was considered the gold standard for diagnosis. A receiver operating characteristic (ROC) analysis was performed to select the cut-off values on the basis of the best tradeoff values between sensitivity and specificity. Results A total of 157 patients were included, 47 (29.9%) with clinical confirmation of GCA after 6 months. 41 (87.2%) of patients with GCA had positive US findings (61.7% had cranial and 44.7% extracranial involvement). The best threshold IMT values were 0.44 mm for the common temporal artery; 0.34 mm for the frontal branch; 0.36 mm for the parietal branch; 1.1 mm for the carotid artery and 1 mm for the subclavian and axillary arteries. The areas under the ROC curves were greater for axillary arteries 0.996 (95% CI 0.991-1), for parietal branch 0.991 (95% CI 0.980-1), for subclavian 0.990 (95% CI 0.979-1), for frontal branch 0.989 (95% CI 0.976-1), for common temporal artery 0.984 (95% CI 0.959-1) and for common carotid arteries 0.977 (95% CI 0.961-0.993). Conclusion IMT cut-off values have been identified for each artery. These proposed IMT cut-off values may help to improve the diagnostic accuracy of US in clinical practice.
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Affiliation(s)
- Katerine López-Gloria
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Isabel Castrejón
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Juan Carlos Nieto-González
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Pablo Rodríguez-Merlos
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Belén Serrano-Benavente
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Carlos Manuel González
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Indalecio Monteagudo Sáez
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Teresa González
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - José María Álvaro-Gracia
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Juan Molina-Collada
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
- Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
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13
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Subclinical giant cell arteritis in new onset polymyalgia rheumatica A systematic review and meta-analysis of individual patient data. Semin Arthritis Rheum 2022; 55:152017. [DOI: 10.1016/j.semarthrit.2022.152017] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2022] [Revised: 03/25/2022] [Accepted: 04/25/2022] [Indexed: 01/18/2023]
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14
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Molina-Collada J, Castrejón I, Rivera J, Martínez-Barrio J, Nieto-González JC, López K, Montero F, Trives L, González C, Álvaro-Gracia JM. The Role of Ultrasound and FDG-PET/CT to Detect Extracranial Artery Involvement in Patients with Suspected Large Vessel Vasculitis. Mod Rheumatol 2022; 33:549-556. [PMID: 35661221 DOI: 10.1093/mr/roac058] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2022] [Revised: 05/16/2022] [Accepted: 06/02/2022] [Indexed: 11/12/2022]
Abstract
OBJECTIVE To assess the accuracy of ultrasound (US) versus FDG-PET/CT to identify extracranial involvement in suspected large vessel vasculitis (LVV) patients. METHODS Retrospective observational study of patients referred to our US fast track clinic with suspected LVV. All patients underwent US exam within 24 hours per protocol. FDG-PET/CT was performed according to clinician criteria. The gold standard for LVV diagnosis was clinical confirmation after 6 months. RESULTS Of the 113 patients included (74.3% female, mean age 74 years), 37(32.7%) were diagnosed of LVV after 6 months. Sensitivity and specificity of US was 86.5% and 96.1%, respectively (85.7% and 87.1% for intracranial and 94.1% and 80.2% for extracanial arteries, respectively). Only 12(42.9%) of 28 patients undergoing a FDG-PET/CT per clinician criteria, showed positive findings. Sensitivity and specificity of FDG-PET/CT for LVV was 61.1% and 90%, respectively (55.6% and 63.2% for intracranial and 84.6% and 93.3% for extacranial arteries). Taking FDG-PET/CT as the reference, US showed extracranial inflammation in 10/12(83.3%) and detected 2(12.5%) additional cases of extracranial involvement with negative FDG-PET/CT. Conversely, FDG-PET/CT showed extracranial inflammation in 2 patients with negative US (1 isolated aortitis and 1 aortoiliac involvement). FDG-PET/CT showed cranial artery uptake in none of patients. CONCLUSIONS US and FDG-PET/CT are both valid tools to detect extracranial involvement. Presence of US extracranial artery inflammation is consistent with FDG-PET/CT examination, although a negative US scan does not rule out extracranial involvement. Our data support the use of US as first-line investigation in patients suspected of cranial, but also extracranial LVV.
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Affiliation(s)
- Juan Molina-Collada
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Isabel Castrejón
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Javier Rivera
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Julia Martínez-Barrio
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Juan Carlos Nieto-González
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Katerine López
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Fernando Montero
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Laura Trives
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - Carlos González
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
| | - José María Álvaro-Gracia
- Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.,Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain
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15
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Nagarajah R, Gupta R, Kumar S. Diagnostic use of ultrasound in giant cell arteritis in Counties Manukau district health board, New Zealand. Rheumatol Adv Pract 2022; 6:rkac040. [PMID: 35663155 PMCID: PMC9154064 DOI: 10.1093/rap/rkac040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2021] [Accepted: 04/23/2022] [Indexed: 11/25/2022] Open
Abstract
Objectives A retrospective observational study was undertaken to assess the diagnostic performance (sensitivity and specificity) of colour duplex ultrasound (CDUS) compared with temporal artery biopsy (TAB) for the diagnosis of GCA in the Counties Manukau District Health Board (CMDHB), New Zealand using clinical diagnosis as the reference standard. Methods The study population included patients with clinically suspected GCA who were referred to Middlemore Hospital and underwent CDUS, TAB or both between January 2019 and December 2020. Results Sixty-nine patients were included in the study. Sixty-one percent were >75 years of age, with no cases <50 years of age and a female predominance of 71%. The sensitivity of CDUS was 26% (95% CI 10, 48) and specificity was 97% (95% CI 84, 100). The sensitivity of TAB was 57% (95% CI 34, 77) and specificity was 100%. CDUS had a positive predictive value of 86% (95% CI 42, 99) and a negative predictive value of 65% (95% CI 49, 78). Conclusion A positive CDUS in patients with a high risk for GCA may preclude the need for TAB due to the high specificity of CDUS in GCA. In contrast, patients with a high risk for GCA with a negative CDUS may still need TAB to confirm or exclude GCA. The duration from commencement of steroids to the time of CDUS is crucial in confirming GCA and, for this, shortening the waiting time in the CMDHB would be necessary to ensure adequate test performance in practice.
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Affiliation(s)
- Rathan Nagarajah
- Department of Internal medicine, Middlemore Hospital, Auckland, New Zealand
| | - Rajiv Gupta
- Department of Rheumatology, Middlemore Hospital, Auckland, New Zealand
| | - Sunil Kumar
- Department of Rheumatology, Middlemore Hospital, Auckland, New Zealand
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16
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Das S, Goswami RP, Sinha D, Ghosal A, Datta A, Sarkar S. Efficacy of leflunomide as a steroid‐sparing agent in treatment of Indian giant cell arteritis patients: A 2‐year follow‐up study. Int J Rheum Dis 2022; 25:650-658. [DOI: 10.1111/1756-185x.14319] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2021] [Revised: 03/12/2022] [Accepted: 03/18/2022] [Indexed: 12/27/2022]
Affiliation(s)
- Shyamashis Das
- Department of Rheumatology Institute of Neurosciences Kolkata (I‐NK) Kolkata India
| | - Rudra Prosad Goswami
- Department of Rheumatology All India Institute of Medical Sciences New Delhi India
| | - Debanjali Sinha
- Department of Rheumatology Institute of Neurosciences Kolkata (I‐NK) Kolkata India
| | - Anirban Ghosal
- Department of Neurology Institute of Neurosciences Kolkata (I‐NK) Kolkata India
| | - Ashis Datta
- Department of Neurology Institute of Neurosciences Kolkata (I‐NK) Kolkata India
| | - Sanjukta Sarkar
- Department of Radiology Institute of Neurosciences Kolkata (I‐NK) Kolkata India
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17
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Ughi N, Padoan R, Crotti C, Sciascia S, Carrara G, Zanetti A, Rozza D, Monti S, Camellino D, Muratore F, Emmi G, Quartuccio L, Morbelli S, El Aoufy K, Tonolo S, Caporali R, De Vita S, Salvarani C, Cimmino M. The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis. Reumatismo 2022; 73. [DOI: 10.4081/reumatismo.2021.1470] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2021] [Accepted: 01/24/2022] [Indexed: 11/23/2022] Open
Abstract
Objective: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations. Methods: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients’ representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations. Results: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids. Conclusions: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.
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18
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Bilici S, Duman R. The current clinical role of optical coherence tomography angiography in neuro-ophthalmological diseases. Taiwan J Ophthalmol 2022; 12:264-272. [PMID: 36248087 PMCID: PMC9558467 DOI: 10.4103/tjo.tjo_55_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2021] [Accepted: 12/01/2021] [Indexed: 11/04/2022] Open
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19
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Micieli JA, Grossniklaus HE, Peragallo JH. Masquerades of Giant Cell Arteritis Evident on Temporal Artery Biopsy. J Clin Rheumatol 2021; 27:e568-e574. [PMID: 31124922 DOI: 10.1097/rhu.0000000000001072] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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20
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Ponte C, Monti S, Scirè CA, Delvino P, Khmelinskii N, Milanesi A, Teixeira V, Brandolino F, Saraiva F, Montecucco C, Fonseca JE, Schmidt WA, Luqmani RA. Ultrasound halo sign as a potential monitoring tool for patients with giant cell arteritis: a prospective analysis. Ann Rheum Dis 2021; 80:1475-1482. [PMID: 34215646 DOI: 10.1136/annrheumdis-2021-220306] [Citation(s) in RCA: 39] [Impact Index Per Article: 9.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2021] [Accepted: 06/16/2021] [Indexed: 12/15/2022]
Abstract
OBJECTIVES To assess the sensitivity to change of ultrasound halo features and their association with disease activity and glucocorticoid (GC) treatment in patients with newly diagnosed giant cell arteritis (GCA). METHODS Prospective study of patients with ultrasound-confirmed GCA who underwent serial ultrasound assessments of the temporal artery (TA) and axillary artery (AX) at fixed time points. The number of segments with halo and maximum halo intima-media thickness (IMT) was recorded. Time points in which >80% of patients were assessed were considered for analysis. Halo features at disease presentation and first relapse were compared. RESULTS 49 patients were assessed at 354 visits. Halo sensitivity to change was assessed at weeks 1, 3, 6, 12 and 24 and showed a significant standardised mean difference between all time points and baseline for the TA halo features but only after week 6 for the AX halo features. The number of TA segments with halo and sum and maximum TA halo IMT showed a significant correlation with erythrocyte sedimentation rate (0.41, 0.44 and 0.48), C reactive protein (0.34, 0.39 and 0.41), Birmingham Vasculitis Activity Score (0.29, 0.36 and 0.35) and GC cumulative dose (-0.34, -0.37 and -0.32); no significant correlation was found for the AX halo features. Halo sign was present in 94% of first disease relapses but with a lower mean number of segments with halo and sum of halo IMT compared with disease onset (2.93±1.59 mm vs 4.85±1.51 mm, p=0.0012; 2.01±1.13 mm vs 4.49±1.95 mm, p=0.0012). CONCLUSIONS Ultrasound is a useful imaging tool to assess disease activity and response to treatment in patients with GCA.
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Affiliation(s)
- Cristina Ponte
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Portugal
- Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
| | - Sara Monti
- Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
- PhD in Experimental Medicine, University of Pavia, Pavia, Italy
| | - Carlo Alberto Scirè
- Epidemiology Research Unit, Italian Society of Rheumatology, Milano, Italy
- Department of Medical Sciences, Rheumatology Unit, University of Ferrara, Ferrara, Italy
| | - Paolo Delvino
- Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | - Nikita Khmelinskii
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Portugal
- Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
| | - Alessandra Milanesi
- Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | - Vítor Teixeira
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Portugal
- Rheumatology Department, Centro Hospitalar Universitário do Algarve, Faro, Portugal
| | - Fabio Brandolino
- Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | - Fernando Saraiva
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Portugal
| | | | - João Eurico Fonseca
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Portugal
- Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
| | - Wolfgang A Schmidt
- Medical Centre for Rheumatology Berlin-Buch, Immanuel Krankenhaus Berlin, Berlin, Germany
| | - Raashid Ahmed Luqmani
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK
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Sebastian A, Coath F, Innes S, Jackson J, van der Geest KSM, Dasgupta B. Role of the halo sign in the assessment of giant cell arteritis: a systematic review and meta-analysis. Rheumatol Adv Pract 2021; 5:rkab059. [PMID: 34514295 PMCID: PMC8421813 DOI: 10.1093/rap/rkab059] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2021] [Accepted: 08/09/2021] [Indexed: 02/06/2023] Open
Abstract
OBJECTIVES This systematic review and meta-analysis aimed to evaluate the diagnostic value of the halo sign in the assessment of GCA. METHODS A systematic literature review was performed using MEDLINE, EMBASE and Cochrane central register databases up to August 2020. Studies informing on the sensitivity and specificity of the US halo sign for GCA (index test) were selected. Studies with a minimum of five participants were included. Study articles using clinical criteria, imaging such as PET-CT and/or temporal artery biopsy (TAB) as the reference standards were selected. Meta-analysis was conducted with a bivariate model. RESULTS The initial search yielded 4023 studies. Twenty-three studies (patients n = 2711) met the inclusion criteria. Prospective (11 studies) and retrospective (12 studies) studies in academic and non-academic centres were included. Using clinical diagnosis as the standard (18 studies) yielded a pooled sensitivity of 67% (95% CI: 51, 80) and a specificity of 95% (95% CI: 89, 98%). This gave a positive and negative likelihood ratio for the diagnosis of GCA of 14.2 (95% CI: 5.7, 35.5) and 0.375 (95% CI: 0.22, 0.54), respectively. Using TAB as the standard (15 studies) yielded a pooled sensitivity of 63% (95% CI: 50, 75) and a specificity of 90% (95% CI: 81, 95). CONCLUSION The US halo sign is a sensitive and specific approach for GCA assessment and plays a pivotal role in diagnosis of GCA in routine clinical practice. REGISTRATION PROSPERO 2020 CRD42020202179.
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Affiliation(s)
- Alwin Sebastian
- Department of Rheumatology, Southend University Hospital, Mid and South Essex University Hospital Groups, Westcliff-On-Sea
- School of Sport, Rehabilitation and Exercise Sciences, University of Essex, Colchester, Essex
| | - Fiona Coath
- Department of Rheumatology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, Norfolk, UK
| | - Sue Innes
- School of Sport, Rehabilitation and Exercise Sciences, University of Essex, Colchester, Essex
| | - Jo Jackson
- School of Sport, Rehabilitation and Exercise Sciences, University of Essex, Colchester, Essex
| | - Kornelis S M van der Geest
- Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
| | - Bhaskar Dasgupta
- Department of Rheumatology, Southend University Hospital, Mid and South Essex University Hospital Groups, Westcliff-On-Sea
- School of Sport, Rehabilitation and Exercise Sciences, University of Essex, Colchester, Essex
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Coath FL, Mukhtyar C. Ultrasonography in the diagnosis and follow-up of giant cell arteritis. Rheumatology (Oxford) 2021; 60:2528-2536. [PMID: 33599253 DOI: 10.1093/rheumatology/keab179] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2020] [Revised: 02/05/2021] [Accepted: 02/08/2021] [Indexed: 12/22/2022] Open
Abstract
Colour Doppler ultrasonography is the first measure to allow objective bedside assessment of GCA. This article discusses the evidence using the OMERACT filter. Consensus definitions for ultrasonographic changes were agreed upon by a Delphi process, with the 'halo' and 'compression' signs being characteristic. The halo is sensitive to change, disappearing within 2-4 weeks of starting glucocorticoids. Ultrasonography has moderate convergent validity with temporal artery biopsy in a pooled analysis of 12 studies including 965 participants [κ = 0.44 (95% CI 0.38, 0.50)]. The interobserver and intra-observer reliabilities are good (κ = 0.6 and κ = 0.76-0.78, respectively) in live exercises and excellent when assessing acquired images and videos (κ = 0.83-0.87 and κ = 0.88, respectively). Discriminant validity has been tested against stroke and diabetes mellitus (κ=-0.16 for diabetes). Machine familiarity and adequate examination time improves performance. Ultrasonography in follow-up is not yet adequately defined. Some patients have persistent changes in the larger arteries but these do not necessarily imply treatment failure or predict relapses.
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Affiliation(s)
- Fiona L Coath
- Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
| | - Chetan Mukhtyar
- Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
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Hoffman MA, Ekladious A. Clinically isolated descending thoracic aortitis in a healthy older woman: a diagnostic challenge. BMJ Case Rep 2021; 14:e238009. [PMID: 33602759 PMCID: PMC7896571 DOI: 10.1136/bcr-2020-238009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/21/2020] [Indexed: 11/04/2022] Open
Abstract
A woman travelling to Australia in her early 70s presents to a regional emergency department with chest pain and associated shortness of breath. Her medical history was that of seasonal affective disorder treated with citalopram, and an allergy to ibuprofen. Subsequent CT imaging revealed aortic wall thickening and associated periaortic fluid, and a moderate pleural effusion. This was successfully treated with oral prednisolone, responding within 1 day. Further blood tests revealed a high CD4/CD8 T-cell ratio, which can be seen in autoimmune disease, sarcoidosis and haematological malignancies. Without evidence for other autoimmune processes, the patient was given a provisional diagnosis of descending thoracic aortitis secondary to sarcoidosis, prescribed a weaning regimen of prednisolone, and asked to seek further investigation and management in her home country. This is a case with several learning points; rare disease can cause common presentations/reports, and sometimes empirical therapy is the only therapy.
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Affiliation(s)
- Max Amos Hoffman
- Faculty of Health and Medical Sciences, The University of Western Australia, Perth, Western Australia, Australia
| | - Adel Ekladious
- Faculty of Health and Medical Sciences, The University of Western Australia, Perth, Western Australia, Australia
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Navahi RAA, Chaibakhsh S, Alemzadeh SA, Aghdam KA. The Adequate Number of Histopathology Cross-sections of Temporal Artery Biopsy in Establishing the Diagnosis of Giant Cell Arteritis. J Ophthalmic Vis Res 2021; 16:77-83. [PMID: 33520130 PMCID: PMC7841273 DOI: 10.18502/jovr.v16i1.8253] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2019] [Accepted: 09/22/2020] [Indexed: 11/24/2022] Open
Abstract
Purpose To determine the appropriate number of histopathological cross-sections that are required for a conclusive diagnosis of giant cell arteritis (GCA). Methods In this cross-sectional study, the number of sections per slide for paraffin-embedded blocks for 100 randomly selected cases where GCA was suspected and those for negative temporal artery biopsies (TABs) were compared with the number of cross-sections per specimen for eight positive-TABs. All aforementioned examinations were conducted at our center from 2012 to 2016. Then, negative-TABs were retrieved and re-evaluated using light microscopy considering the histopathological findings of GCA. Results Ninety-five paraffin blocks were retrieved. The original mean biopsy length was 15.39 ± 7.56 mm. Comparison of the mean number of cross-sections per specimen for both the positive- and negative-TABs (9.25 ± 3.37 and 9.53 ± 2.46) showed that 9.87 ± 2.77 [95% confidence intervals (CI)] cross-sections per specimen were sufficient for a precise GCA diagnosis. There was no statistically significant difference in the mean biopsy length (P = 0.142) among the eight positive-TABs. Similarly, no significant difference was observed in the number of cross-sections per specimen (P = 0.990) for positive-TABs compared to those for the negative-TABs. After the retrieval of negative-TABs, the mean number of total pre- and post-retrieval cross-sections per specimen was 17.66 ± 4.43. Among all retrieved specimens, only one case (0.01%) showed the histopathological features of healed arteritis. Conclusion Positive-TABs did not reveal more histological cross-sections than the negative ones and increasing the number of cross-sections did not enhance the accuracy of TAB.
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Affiliation(s)
- Roshanak Ali-Akbar Navahi
- Eye Research Center, The Five Senses Institute, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
| | - Samira Chaibakhsh
- Eye Research Center, The Five Senses Institute, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
| | - Sayyed Amirpooya Alemzadeh
- Eye Research Center, The Five Senses Institute, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
| | - Kaveh Abri Aghdam
- Eye Research Center, The Five Senses Institute, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
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Noumegni SR, Hoffmann C, Cornec D, Gestin S, Bressollette L, Jousse-Joulin S. Temporal Artery Ultrasound to Diagnose Giant Cell Arteritis: A Practical Guide. ULTRASOUND IN MEDICINE & BIOLOGY 2021; 47:201-213. [PMID: 33143971 DOI: 10.1016/j.ultrasmedbio.2020.10.004] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/16/2020] [Revised: 10/05/2020] [Accepted: 10/06/2020] [Indexed: 06/11/2023]
Abstract
The diagnostic modalities for giant cell arteritis (GCA) have evolved significantly in recent years. Among the different diagnostic tools developed, Doppler ultrasound of the temporal arteries, with a sensitivity and specificity reaching 69% and 82%, respectively, is now recognized as superior and, therefore, is a first-line diagnostic tool in GCA. Moreover, with the increasing development of new ultrasound technologies, the accuracy of Doppler ultrasound in GCA seems to be constantly improving. In this article, we describe in detail the scanning technique to perform while realizing Doppler ultrasound of temporal arteries to assess GCA, as well as the diagnostic performance of this tool according to current literature.
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Affiliation(s)
- Steve Raoul Noumegni
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, Brest, France.
| | - Clément Hoffmann
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, Brest, France
| | - Divi Cornec
- Rheumatology Department, Brest Teaching Hospital, Brest University, Brest, France
| | - Simon Gestin
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, Brest, France
| | - Luc Bressollette
- Vascular Medicine Department, Brest Teaching Hospital, Brest University, Brest, France
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Clinical, Laboratory and Ultrasonographic Interrelations in Giant Cell Arteritis. J Stroke Cerebrovasc Dis 2021; 30:105601. [PMID: 33497936 DOI: 10.1016/j.jstrokecerebrovasdis.2021.105601] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2020] [Accepted: 01/04/2021] [Indexed: 11/20/2022] Open
Abstract
OBJECTIVES The diagnosis of giant cell arteritis (GCA) is based on the presence of clinical and laboratory features. Color-duplex sonography (CDS) may supplant the limited sensitivity of temporal artery biopsy. The aim of our work was to characterize clinical and laboratory findings in patients with positive CDS for GCA. MATERIALS AND METHODS Retrospective study of all consecutive patients of our center fulfilling American College of Rheumatology criteria for GCA who performed CDS study between 2009-2019. Data on clinical and laboratory features were compared in two groups: with and without halo sign. RESULTS Ninety-one patients were included. Temporal halo sign was identified in 46% of patients. Halo sign was more often present in older patients (77 ± 8 vs 73 ± 8 years, p = 0.022), associated with systemic features (58% vs 42%, p = 0.011), higher erythrocyte sedimentation rate (84 ± 26 vs 74 ± 34 mm/hour, p = 0.020), and lower hemoglobin values (10.9 ± 1.5 vs 12.1 ± 1.6 g/dL, p < 0.001). The number of patients under corticosteroids before CDS was higher in the group without halo (62% vs 33%, p = 0.005). Ischemic stroke occurred in 17 patients (19%), 76% in the vertebrobasilar territory, and stroke was associated with vertebral halo sign (p < 0.001). CONCLUSIONS Halo sign was present in half of our patients. Previous corticosteroids treatment decreased positive CDS findings. Systemic symptoms and laboratory findings are more notorious in halo sign subgroup of patients. Stroke cases in GCA patients disproportionally affected the posterior circulation. Ultrasonography provides information about a more pronounced systemic involvement and a higher risk of major complications.
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Simon S, Ninan J, Hissaria P. Diagnosis and management of giant cell arteritis: Major review. Clin Exp Ophthalmol 2021; 49:169-185. [PMID: 33426764 DOI: 10.1111/ceo.13897] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2020] [Revised: 12/06/2020] [Accepted: 12/13/2020] [Indexed: 12/13/2022]
Abstract
Giant cell arteritis is a medical emergency because of the high risk of irreversible blindness and cerebrovascular accidents. While elevated inflammatory markers, temporal artery biopsy and modern imaging modalities are useful diagnostic aids, thorough history taking and clinical acumen still remain key elements in establishing a timely diagnosis. Glucocorticoids are the cornerstone of treatment but are associated with high relapse rates and side effects. Targeted biologic agents may open up new treatment approaches in the future.
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Affiliation(s)
- Sumu Simon
- Department of Ophthalmology and South Australian Institute of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
| | - Jem Ninan
- Department of Rheumatology, Modbury Public Hospital, Modbury, South Australia, Australia
| | - Pravin Hissaria
- Department of Immunology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
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Subclavian artery involvement in patients with giant cell arteritis: do we need a modified Halo Score? Clin Rheumatol 2021; 40:2821-2827. [PMID: 33432449 DOI: 10.1007/s10067-020-05577-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2020] [Revised: 12/16/2020] [Accepted: 12/29/2020] [Indexed: 10/22/2022]
Abstract
OBJECTIVE To assess whether adding the subclavian artery examination into the ultrasound (US) Southend Halo Score, as proposed in the modified Halo Score, improves the diagnostic accuracy of giant cell arteritis (GCA) and its relationship with systemic inflammation. METHODS Retrospective observational study of patients referred to a GCA fast track pathway (FTP) over a 1-year period. Patients underwent US exam of temporal and large vessel (LV) (carotid, subclavian, and axillary) arteries. The extent of inflammation was measured by the halo count, the Southend Halo Score, and the modified Halo Score. The gold standard for GCA diagnosis was clinical confirmation after 6-month follow-up. RESULTS Sixty-four patients were evaluated in the FTP, 17 (26.5%) had GCA. Subclavian artery involvement was present only in patients with GCA (29.4% versus 0%, p < 0.001). Overall, the three scores showed excellent diagnostic accuracy for GCA (ROC AUC 0.906, 0.930, and 0.928, respectively) and moderate correlations with acute phase reactants (0.35-0.51, p < 0.01). Only the modified Halo Score correlated with markers of inflammation in patients with LV involvement. CONCLUSIONS The inclusion of subclavian artery examination in the modified Halo Score does not improve the diagnostic accuracy of GCA. Nevertheless, it correlates better with markers of systemic inflammation in LV-GCA. Key Points • Adding the subclavian artery examination into the Southend Halo Score, as proposed in the modified Halo Score, does not improve the diagnostic accuracy of GCA. • However, the extent of vascular inflammation as quantified by the modified Halo Score correlates better with markers of systemic inflammation in the large vessel (LV) GCA subgroup of patients. • Although the diagnostic value of adding subclavian arteries to the current recommended US examination of GCA is limited, it may have a role in monitoring disease activity as it correlates with the general burden of inflammation in LV GCA. These findings need to be confirmed in additional populations and larger prospective studies.
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Psychogios K, Magoufis G, Kargiotis O, Safouris A, Bakola E, Chondrogianni M, Zis P, Stamboulis E, Tsivgoulis G. Ultrasound Assessment of Extracranial Carotids and Vertebral Arteries in Acute Cerebral Ischemia. ACTA ACUST UNITED AC 2020; 56:medicina56120711. [PMID: 33353035 PMCID: PMC7765801 DOI: 10.3390/medicina56120711] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2020] [Revised: 12/09/2020] [Accepted: 12/16/2020] [Indexed: 12/25/2022]
Abstract
Assessing ischemic etiology and mechanism during the acute phase of an ischemic stroke is crucial in order to tailor and monitor appropriate treatment and determine prognosis. Cervical Duplex Ultrasound (CDU) has evolved since many years as an excellent screening tool for the evaluation of extracranial vasculature. CDU has the advantages of a low cost, easily applicable, bed side examination with high temporal and spatial resolution and without exposing the patients to any significant complications. It represents an easily repeatable test that can be performed in the emergency room as a first-line examination of cervical artery pathology. CDU provides well validated estimates of the type of the atherosclerotic plaque, the degree of stenosis, as well as structural and hemodynamic information directly about extracranial vessels (e.g., subclavian steal syndrome) and indirectly about intracranial circulation. CDU may also aid the diagnosis of non-atherosclerotic lesions of vessel walls including dissections, arteritis, carotid-jugular fistulas and fibromuscular dysplasias. The present narrative review outlines all potential applications of CDU in acute stroke management and also highlights its potential therapeutic implications.
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Affiliation(s)
- Klearchos Psychogios
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
- Second Department of Neurology, Attikon University Hospital, 15772 Athens, Greece; (E.B.); (M.C.)
- School of Medicine, University of Athens, 15772 Athens, Greece
| | - Georgios Magoufis
- Department of Interventional Neuroradiology, Metropolitan Hospital, 18547 Piraeus, Greece;
| | - Odysseas Kargiotis
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
| | - Apostolos Safouris
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
| | - Eleni Bakola
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
| | - Maria Chondrogianni
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
| | - Panagiotis Zis
- Medical School, University of Cyprus, 1678 Nicosia, Cyprus;
| | - Elefterios Stamboulis
- Acute Stroke Unit, Metropolitan Hospital, 18547 Piraeus, Greece; (K.P.); (O.K.); (A.S.); (E.S.)
| | - Georgios Tsivgoulis
- Second Department of Neurology, Attikon University Hospital, 15772 Athens, Greece; (E.B.); (M.C.)
- Correspondence:
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Estudio comparativo de la ecografía Doppler frente a la biopsia de arteria temporal en el diagnóstico de la arteritis de células gigantes. ACTA ACUST UNITED AC 2020; 16:313-318. [DOI: 10.1016/j.reuma.2018.08.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2018] [Revised: 07/27/2018] [Accepted: 08/15/2018] [Indexed: 11/24/2022]
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Rubenstein E, Maldini C, Gonzalez-Chiappe S, Chevret S, Mahr A. Sensitivity of temporal artery biopsy in the diagnosis of giant cell arteritis: a systematic literature review and meta-analysis. Rheumatology (Oxford) 2020; 59:1011-1020. [PMID: 31529073 DOI: 10.1093/rheumatology/kez385] [Citation(s) in RCA: 81] [Impact Index Per Article: 16.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2019] [Revised: 07/30/2019] [Indexed: 12/21/2022] Open
Abstract
OBJECTIVES Temporal artery biopsy (TAB) is a reference test for the diagnosis of GCA but reveals inflammatory changes only in a subset of patients. The lack of knowledge of TAB sensitivity hampers comparisons with non-invasive techniques such as temporal artery ultrasonography. We performed a systematic literature review and meta-analysis to estimate the sensitivity of TAB in GCA and to identify factors that may influence the estimate. METHODS A systematic literature review involved searching electronic databases and cross-references. Eligibility criteria included publications reporting at least 30 GCA cases fulfilling the original or modified 1990 ACR classification criteria. The pooled proportion of TAB-positive GCA cases was calculated by using aggregated-data meta-analysis with a random-effects model and assessment of heterogeneity with the I2 statistic. Subgroup analyses and meta-regression were used to examine the effect of patient and study characteristics on TAB positivity. RESULTS Among 3820 publications screened, 32 studies (3092 patients) published during 1993-2017 were analysed. The pooled proportion of TAB-positive GCA cases was 77.3% (95% CI: 71.8, 81.9%), with high between-study heterogeneity (I2 = 90%). The proportion of TAB-positive cases was slightly higher in publications before than in 2012 and after (P = 0.001). CONCLUSION The estimated sensitivity of 77% provides indirect evidence that TAB is not less sensitive than temporal artery imaging. The unexplained high between-study heterogeneity could result from differences in TAB sampling, processing or interpretation. The decrease in TAB-positive GCA cases over time could reflect an increasing propensity for clinicians to accept a GCA diagnosis without proof by TAB.
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Affiliation(s)
- Emma Rubenstein
- Department of Internal Medicine, Hospital Saint-Louis, University Paris Diderot
| | - Carla Maldini
- Department of Internal Medicine, Hospital Saint-Louis, University Paris Diderot
| | | | - Sylvie Chevret
- Department of Internal Medicine, Hospital Saint-Louis, University Paris Diderot
| | - Alfred Mahr
- Department of Internal Medicine, Hospital Saint-Louis, University Paris Diderot
- ECSTRRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, Paris, France
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Sebastian A, van der Geest KSM, Coath F, Gondo P, Kayani A, Mackerness C, Hadebe B, Innes S, Jackson J, Dasgupta B. Halo score (temporal artery, its branches and axillary artery) as a diagnostic, prognostic and disease monitoring tool for Giant Cell Arteritis (GCA). BMC Rheumatol 2020; 4:35. [PMID: 32821876 PMCID: PMC7433165 DOI: 10.1186/s41927-020-00136-5] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2020] [Accepted: 05/03/2020] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Giant cell arteritis (GCA) is a common large vessel vasculitis of the elderly, often associated with sight loss. Glucocorticoids (GC remain the mainstay of treatment, although biologic treatments have been approved. Biomarkers predicting disease severity, relapse rates and damage are lacking in GCA.EULAR recommends ultrasound (US) as the first investigation for suspected GCA. The cardinal US finding, a non-compressible halo, is currently categorised as either negative or positive. However, the extent and severity of this finding may vary.In this study, we hypothesise whether the extent and severity of the halo sign [calculated as a single composite Halo score (HS)] of temporal and axillary arteries may be of diagnostic, prognostic and monitoring importance; whether baseline HS is linked to disease outcomes, relapses and damage; whether HS can stratify GCA patients for individual treatment needs; whether HS can function as an objective monitoring tool during follow up. METHODS This is a prospective, observational study. Suspected GCA Participants will be selected from the GCA FTC at the participating centres in the UK. Informed consent will be obtained, and patients managed as part of standard care. Patients with GCA will have HS (temporal and axillary arteries) measured at baseline and months 1,3,6 and 12 long with routine clinical assessments, blood sampling and patient-reported outcomes (EQ5D). Non-GCA patients will be discharged back to the referral team and will have a telephone interview in 6 months.We aim to recruit 272 suspected GCA referrals which should yield 68 patients (25% of referrals) with confirmed GCA. The recruitment will be completed in 1 year with an estimated total study period of 24 months. DISCUSSION The identification of prognostic factors in GCA is both timely and needed. A prognostic marker, such as the HS, could help to stratify GCA patients for an appropriate treatment regimen. Tocilizumab, an IL-6R blocking agent, switches off the acute phase response (C-Reactive Protein), making it difficult to measure the disease activity. Therefore, an independent HS, and changes in that score during treatment and follow-up, maybe a more objective measure of response compare to patient-reported symptoms and clinical assessment alone.
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Affiliation(s)
- Alwin Sebastian
- Rheumatology, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
- University of Essex, Colchester, UK
| | - Kornelis S. M. van der Geest
- Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Fiona Coath
- Norfolk and Norwich University hospitals NHS Foundation Trust, Colney Ln, Norwich, UK
| | - Prisca Gondo
- R&D, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
| | - Abdul Kayani
- Rheumatology, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
| | - Craig Mackerness
- R&D, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
| | - Bernard Hadebe
- R&D, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
| | - Sue Innes
- School of Sport, Rehabilitation and exercise sciences, Colchester campus, University of Essex, Colchester, UK
| | - Jo Jackson
- School of Sport, Rehabilitation and exercise sciences, Colchester campus, University of Essex, Colchester, UK
| | - Bhaskar Dasgupta
- Rheumatology, Mid and South Essex University Hospital Groups, Southend University Hospital, Westcliff-On-Sea, Essex, UK
- University of Essex, Colchester, UK
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Thomassen I, Brok AND, Konings CJ, Nienhuijs SW, Van De Poll MCG. Steroid Use is Associated with Clinically Irrelevant Biopsies in Patients with Suspected Giant Cell Arteritis. Am Surg 2020. [DOI: 10.1177/000313481207801228] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Temporal artery biopsy (TAB) is the diagnostic gold standard for giant cell arteritis (GCA). GCA is treated by high-dose corticosteroids. In cases of high clinical suspicion, steroids may be administrated despite negative TAB, making TAB clinically irrelevant. We assessed the role of TAB in clinical decision-making in patients with suspected GCA and to identify factors associated with clinically irrelevant TAB. Charts of patients who underwent TAB from 2005 to 2010 were reviewed for clinical parameters potentially associated with GCA and clinically irrelevant TAB. We studied 143 patients with 99 negative (69%), 34 positive (24%), and 10 undefined (7%) TABs. Eventually 26 patients (18% of the entire cohort and 26% of the patients with a negative TAB) received steroid treatment for GCA despite negative TAB. The start of steroid treatment before TAB was associated with clinically irrelevant TABs. If clinical suspicion of GCA is high, a TAB can be considered clinically irrelevant.
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Affiliation(s)
- Irene Thomassen
- Departments of Surgery, Catharina Hospital Eindhoven, Eindhoven, The Netherlands
| | - Avalon N. Den Brok
- Departments of Internal Medicine, Catharina Hospital Eindhoven, Eindhoven, The Netherlands
| | | | - Simon W. Nienhuijs
- Departments of Surgery, Catharina Hospital Eindhoven, Eindhoven, The Netherlands
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Ponte C, Serafim AS, Monti S, Fernandes E, Lee E, Singh S, Piper J, Hutchings A, McNally E, Diamantopoulos AP, Dasgupta B, Schmidt WA, Luqmani RA. Early variation of ultrasound halo sign with treatment and relation with clinical features in patients with giant cell arteritis. Rheumatology (Oxford) 2020; 59:3717-3726. [DOI: 10.1093/rheumatology/keaa196] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Accepted: 03/19/2020] [Indexed: 01/02/2023] Open
Abstract
Abstract
Objectives
To compare the ultrasound characteristics with clinical features, final diagnosis and outcome; and to evaluate the halo size following glucocorticoid treatment in patients with newly diagnosed GCA.
Methods
Patients with suspected GCA, recruited from an international cohort, had an ultrasound of temporal (TA) and axillary (AX) arteries performed within 7 days of commencing glucocorticoids. We compared differences in clinical features at disease presentation, after 2 weeks and after 6 months, according to the presence or absence of halo sign. We undertook a cross-sectional analysis of the differences in halo thickness using Pearson’s correlation coefficient (r) and Analysis of Variance (ANOVA).
Results
A total of 345 patients with 6 months follow-up data were included; 226 (65.5%) had a diagnosis of GCA. Jaw claudication and visual symptoms were more frequent in patients with halo sign (P =0.018 and P =0.003, respectively). Physical examination abnormalities were significantly associated with the presence of ipsilateral halo (P <0.05). Stenosis or occlusion on ultrasound failed to contribute to the diagnosis of GCA. During 7 days of glucocorticoid treatment, there was a consistent reduction in halo size in the TA (maximum halo size per patient: r=−0.30, P =0.001; and all halos r=−0.23, P <0.001), but not in the AX (P >0.05). However, the presence of halo at baseline failed to predict future ischaemic events occurring during follow-up.
Conclusion
In newly diagnosed GCA, TA halo is associated with the presence of ischaemic features and its size decreases following glucocorticoid treatment, supporting its early use as a marker of disease activity, in addition to its diagnostic role.
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Affiliation(s)
- Cristina Ponte
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte
- Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon
| | - Ana Sofia Serafim
- Internal Medicine Department, Centro Hospitalar Barreiro-Montijo, Barreiro, Portugal
| | - Sara Monti
- Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, Pavai
- PhD in Experimental Medicine, University of Pavia, Pavia, Italy
| | - Elisabete Fernandes
- Biomathematics Laboratory, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Ellen Lee
- Clinical Trials Research Unit, ScHARR, The University of Sheffield, Sheffield
| | - Surjeet Singh
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford
| | - Jennifer Piper
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford
| | | | | | | | - Bhaskar Dasgupta
- Department of Rheumatology, Southend Hospital NHS Trust, Westcliff-on-Sea, UK
| | - Wolfgang A Schmidt
- Immanuel Krankenhaus Berlin, Medical Centre for Rheumatology Berlin-Buch, Berlin, Germany
| | - Raashid Ahmed Luqmani
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford
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Ponte C, Martins-Martinho J, Luqmani RA. Diagnosis of giant cell arteritis. Rheumatology (Oxford) 2020; 59:iii5-iii16. [DOI: 10.1093/rheumatology/kez553] [Citation(s) in RCA: 67] [Impact Index Per Article: 13.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/30/2023] Open
Abstract
Abstract
GCA is the most common form of primary systemic vasculitis affecting older people. It is considered a clinical emergency because it can lead to irreversible blindness in around 20% of untreated cases. High doses of glucocorticoids should be initiated promptly to prevent disease-related complications; however, glucocorticoids therapy usually results in significant toxicity. Therefore, correct diagnosis is crucial. For many years, temporal artery biopsy has been considered the diagnostic ‘gold standard’ for GCA, but it has many limitations (including low sensitivity). US has proven to be effective for diagnosing GCA and can reliably replace temporal artery biopsy in particular clinical settings. In cases of suspected GCA with large-vessel involvement, other imaging modalities can be used for diagnosis (e.g. CT and PET). Here we review the current evidence for each diagnostic modality and propose an algorithm to diagnose cranial-GCA in a setting with rapid access to high quality US.
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Affiliation(s)
- Cristina Ponte
- Rheumatology Department, Hospital de Santa Maria – Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon
- Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Joana Martins-Martinho
- Rheumatology Department, Hospital de Santa Maria – Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon
| | - Raashid Ahmed Luqmani
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK
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Dammacco R, Alessio G, Giancipoli E, Leone P, Cirulli A, Resta L, Vacca A, Dammacco F. Giant Cell Arteritis: The Experience of Two Collaborative Referral Centers and an Overview of Disease Pathogenesis and Therapeutic Advancements. Clin Ophthalmol 2020; 14:775-793. [PMID: 32210531 PMCID: PMC7073434 DOI: 10.2147/opth.s243203] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2019] [Accepted: 01/29/2020] [Indexed: 11/23/2022] Open
Abstract
Purpose Giant cell arteritis (GCA), a chronic vasculitis of the large and medium-sized arteries, affects people >50 years of age. This study assessed the prevalence of visual manifestations and other clinical features at presentation in an Italian cohort of GCA patients. Recent advances in the pathophysiology, diagnosis, and therapy of GCA are also reviewed. Methods This retrospective, single-center study conducted by the ophthalmology and internal medicine clinics of one university recruited 56 patients from 2005 to 2016 and followed them for 11-54 months. Results Ocular involvement was diagnosed in 19 patients (33.9%), with permanent vision loss in 19.6% (7.1% of the cohort with bilateral vision loss). Arteritic anterior and posterior ischemic optic neuropathy were diagnosed in 11 patients (57.9%) and 1 patient (5.3%), respectively, cotton wool spots in 3 patients (15.8%), central retinal artery occlusion in 2 patients (10.5%), and anterior segment ischemia and multifocal choroidal ischemia in 1 patient each (5.3%). Polymyalgia rheumatica was associated with GCA in 44.6% of the patients. The most common extra-ocular manifestation was constitutional symptoms (82.1% of the patients). Large-vessel involvement, including of the ascending aorta, aortic arch, and left axillary artery, was diagnosed by magnetic resonance or computed tomography (CT) angiography and 18FDG positron emission/CT. Glucocorticoids (GCs) remain the standard-of-care worldwide, but methotrexate, provided as a steroid-sparing drug in 41% of the patients, resulted in earlier tapering, a lower cumulative dose of GCs, and a lower rate of relapse. Among the combinations of GCs and immunosuppressive drugs proposed to treat GCA, only tocilizumab has effectively induced and maintained disease remission. Conclusion According to our data and literature reports: a) GCA is a systemic disease; b) its diagnosis is expedited by the adjunct use of imaging techniques; c) insights into the pathogenesis of GCA may allow an improved, differentiated therapeutic approach.
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Affiliation(s)
- Rosanna Dammacco
- Department of Ophthalmology and Neuroscience, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Giovanni Alessio
- Department of Ophthalmology and Neuroscience, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Ermete Giancipoli
- Department of Biomedical Sciences, Ophthalmology Unit, University of Sassari, Sassari, Italy
| | - Patrizia Leone
- Department of Biomedical Sciences and Human Oncology, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Anna Cirulli
- Department of Biomedical Sciences and Human Oncology, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Leonardo Resta
- Department of Emergency and Organ Transplantation, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Angelo Vacca
- Department of Biomedical Sciences and Human Oncology, University of Bari "Aldo Moro", Medical School, Bari, Italy
| | - Franco Dammacco
- Department of Biomedical Sciences and Human Oncology, University of Bari "Aldo Moro", Medical School, Bari, Italy
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Membrey B, Miranda S, Lévesque H, Cailleux N, Benhamou Y, Armengol G. Artérite gigantocellulaire : apport de l’écho-doppler. Rev Med Interne 2020; 41:106-110. [DOI: 10.1016/j.revmed.2019.10.337] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Revised: 10/04/2019] [Accepted: 10/21/2019] [Indexed: 01/22/2023]
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Fernández-Fernández E, Monjo-Henry I, Bonilla G, Plasencia C, Miranda-Carús ME, Balsa A, De Miguel E. False positives in the ultrasound diagnosis of giant cell arteritis: some diseases can also show the halo sign. Rheumatology (Oxford) 2020; 59:2443-2447. [DOI: 10.1093/rheumatology/kez641] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2019] [Revised: 11/21/2019] [Indexed: 12/24/2022] Open
Abstract
Abstract
Objectives
To describe the frequency and causes for the presence of a halo sign on the ultrasound of patients without a diagnosis of GCA.
Methods
In total, 305 patients with temporal artery colour Doppler ultrasound showing the presence of halo sign (intima-media thickness ≥0.34 mm for temporal arteries [TAs] and ≥1 mm for axillary arteries) were included, and their medical records were reviewed. The clinical diagnosis based on the evolution of the patient over at least one year was established as the definitive diagnosis.
Results
Fourteen of the 305 (4.6%) patients included showed presence of the halo sign without final diagnosis of GCA: 12 patients in the TAs (86%), and two patients with isolated AAs involvement (14%). Their diagnoses were PMR (n = 4, 29%); atherosclerosis (n = 3, 21%); and non-Hodgkin lymphoma type T, osteomyelitis of the skull base, primary amyloidosis associated with multiple myeloma, granulomatosis with polyangiitis, neurosyphilis, urinary sepsis and narrow-angle glaucoma (n = 1 each, 7%).
Conclusion
The percentage of halo signs on the ultrasound of patients without GCA is low, but it does exist. There are conditions that may also show the halo sign (true positive halo sign), and we must know these and always correlate the ultrasound findings with the patient’s clinic records.
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Affiliation(s)
- Elisa Fernández-Fernández
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Irene Monjo-Henry
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Gema Bonilla
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Chamaida Plasencia
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - María-Eugenia Miranda-Carús
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Alejandro Balsa
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Eugenio De Miguel
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
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Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H, Brouwer E, Cassie R, Cid MC, Dasgupta B, Dejaco C, Hatemi G, Hollinger N, Mahr A, Mollan SP, Mukhtyar C, Ponte C, Salvarani C, Sivakumar R, Tian X, Tomasson G, Turesson C, Schmidt W, Villiger PM, Watts R, Young C, Luqmani RA. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2020; 79:19-30. [PMID: 31270110 DOI: 10.1136/annrheumdis-2019-215672] [Citation(s) in RCA: 670] [Impact Index Per Article: 134.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2019] [Revised: 06/10/2019] [Accepted: 06/10/2019] [Indexed: 12/29/2022]
Abstract
BACKGROUND Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations. METHODS Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations. RESULTS Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons. CONCLUSIONS We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.
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Affiliation(s)
- Bernhard Hellmich
- Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken, University of Tübingen, Kirchheim-Teck, Germany
| | - Ana Agueda
- Rheumatology Department, Centro Hospitalar do Baixo Vouga E.P.E, Aveiro, Portugal
| | - Sara Monti
- Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Frank Buttgereit
- Department of Rheumatology and Immunology, University Hospital Charité, Berlin, Germany
| | - Hubert de Boysson
- Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, Basse-Normandie, France
| | - Elisabeth Brouwer
- Rheumatology and Clinical Immunology, UMCG, Groningen, The Netherlands
| | | | - Maria C Cid
- Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
| | | | - Christian Dejaco
- Rheumatology, Medical University Graz, Graz, Austria
- Rheumatology, Hospital of Bruneck, Bruneck, Italy
| | - Gulen Hatemi
- Division of Rheumatology, Department of Internal Medicine, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey
| | - Nicole Hollinger
- Department of Internal Medicine, Rheumatology and Immunology, Medus Klinken, Karl-Albrechts-Universität Tübingen, Kirchheim-Teck, Germany
| | - Alfred Mahr
- Hospital Saint-Louis, University Paris Diderot, Paris, France
| | - Susan P Mollan
- Ophthalmology, University Hospitals Birmingham, Birmingham, UK
- Neurometabolism, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
| | - Chetan Mukhtyar
- Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
| | - Cristina Ponte
- Rheumatology, Hospital de Santa Maria - CHLN, Lisbon Academic Medical Centre, Lisbon, Portugal
- Rheumatology Research Unit; Instituto de Medicina Molecular, Instituto de Medicina Molecular, Lisboa, Portugal
| | | | - Rajappa Sivakumar
- Stroke and Neurocritical Care, GLB Hospitals and Acute Stroke Centers, Chennai, India
| | - Xinping Tian
- Rheumatology, Peking Union Medical College Hospital, Beijing, China
| | | | - Carl Turesson
- Department of Rheumatology, Skåne University Hospital, Malmö, Sweden
| | - Wolfgang Schmidt
- Medical Centre for Rheumatology Berlin-Buch, Immanuel Krankenhaus Berlin, Berlin, Germany
| | - Peter M Villiger
- Rheumatology and Clinical Immunology / Allerg, University Hospital (Inselspital), Bern, Switzerland
| | - Richard Watts
- Norwich Medical School, Bob Champion Research and Education Building, University of East Anglia, Norwich, UK
| | | | - Raashid Ahmed Luqmani
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, UK
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Will imaging change the diagnosis and management of giant cell arteritis? ACTA ACUST UNITED AC 2019; 57:341-344. [PMID: 31120860 DOI: 10.2478/rjim-2019-0013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2019] [Indexed: 11/20/2022]
Abstract
Giant cell arteritis is a common systemic vasculitis affecting the elderly, with maximum prevalence in the 7th decade of age, targeting aortic derived medium and large vessels of the neck and head. Diagnosis is established on a biopsy specimen of the temporal artery wall, through pathological confirmation of panarteritis, typically characterized by mononuclear cell infiltrate, with the 1990 ACR criteria often used in clinical practice. We present the case of a patient with a new onset headache and systemic inflammation, who did not fulfil the classical diagnostic criteria, nor did the temporal artery biopsy (TAB) provide a positive result. However, the ultrasonographical features, clinical evolution and response to corticosteroid therapy confirmed the diagnosis. This patient had bilateral presence of the halo sign on color duplex ultrasonography (CDUS), cited as a highly specific feature, when compared to the ACR criteria as a standard reference. We employed its positive likelihood-ratio (LR+) of 43 as previously estimated, while considering a low pre-test probability for a positive diagnosis (15%), to calculate a post-test probability of 88%, leading to our decision to treat him as having giant cell arteritis. Remission of the headache and rebound phenomena when tapered off steroid therapy substantially contributed to the positive diagnosis, underlining the importance of future studies needing to use clinical evolution as a reference standard.
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The role of vascular ultrasound in managing giant cell arteritis in ophthalmology. Surv Ophthalmol 2019; 65:218-226. [PMID: 31775013 DOI: 10.1016/j.survophthal.2019.11.004] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2019] [Revised: 11/14/2019] [Accepted: 11/18/2019] [Indexed: 11/20/2022]
Abstract
Giant cell arteritis is the most common systemic vasculitis in the elderly and is a potentially life-threatening ophthalmic emergency that can result in irreversible blindness. Blindness is most commonly associated with acute onset, irreversible arteritic ischemic optic neuropathy. Without treatment, second eye involvement may occur, resulting in bilateral blindness. Patients with established visual loss are treated with high-dose steroids and generally undergo a temporal artery biopsy to confirm their diagnosis. A significant number of patients are, however, referred for urgent ophthalmology assessment from concerns about "incipient" arteritic ischemic optic neuropathy. Before visual loss, patients may experience a range of ocular symptoms related to ischemia. This generally leads to treatment with high-dose systemic steroid and an urgent request for a temporal artery biopsy. Temporal artery biopsy is considered as the standard investigation for confirmatory diagnosis. It is generally arranged as soon as possible, although it is often not carried out for several days, and there may also be delays in histopathological reporting. It is often perceived that the patient is "safe" while on corticosteroids, in that they are being treated to avoid visual loss. What is not acknowledged, however, is that, if patients do not have giant cell arteritis and are being treated "just in case," they will often require a tapering of oral steroids over several weeks, exposing them to unnecessary and significant side effects. In the rheumatology setting, vascular ultrasound has emerged as a safe and reliable alternative to temporal artery biopsy as a point of care diagnostic tool in the management of giant cell arteritis. Given an experienced sonographer and optimal equipment, a rapid diagnosis can be established in a fast-track clinic setting, taking into consideration clinical assessment, scoring, and ultrasound findings. A huge advantage of ultrasound is that it provides immediate information that can be used to inform treatment decisions. We explore the evidence that supports the incorporation of vascular ultrasound into the ophthalmology repertoire to make a more efficient diagnosis that is cost-effective and associated with better patient outcomes, including a potential reduction in loss of sight and avoidance of unnecessary long-term steroid treatment by early exclusion of mimics.
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The Impact of Temporal Artery Biopsy at a UK Tertiary Plastic Surgery Unit. PLASTIC AND RECONSTRUCTIVE SURGERY-GLOBAL OPEN 2019; 7:e2541. [PMID: 31942316 PMCID: PMC6908368 DOI: 10.1097/gox.0000000000002541] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2019] [Accepted: 10/03/2019] [Indexed: 12/30/2022]
Abstract
Guidelines recommend temporal artery biopsy (TAB) for patients suspected of having giant cell arteritis (GCA). We evaluated the impact of TAB on the diagnosis and management of patients with suspected GCA at a tertiary plastic surgery unit. Methods A retrospective review of all TAB procedures performed at our centre over 7 years was performed. One hundred and one patients were included in the study. Patients were classified into 3 diagnostic groups: confirmed (positive TAB), presumed (negative TAB with high clinical suspicion) and unlikely (negative TAB with low clinical suspicion). The clinical presentation and management for each group were compared. Results The average American College of Rheumatology (ACR) score was 3.07. The number of patients with an ACR score of ≥3 before TAB was 72 (71.3%) and remained the same after TAB. The number of patients who remained on steroid therapy was lower in the group with an unlikely diagnosis of GCA compared to the group with a confirmed diagnosis (p<0.05). Conversely, there was no significant difference in steroid therapy between those with a presumed and confirmed diagnosis (p>0.05). Conclusions This study found a significant difference in steroid treatment between those with confirmed GCA and those where the diagnosis was unlikely showing that TAB may support decisions regarding steroid therapy. However, TAB was inappropriately requested for patients whose pre-TAB ACR score was ≥3 as this score is sufficient for the diagnosis of GCA. Therefore, the use of TAB should be limited to cases of diagnostic uncertainty.
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Blockmans D, Luqmani R, Spaggiari L, Salvarani C. Magnetic resonance angiography versus 18F-fluorodeoxyglucose positron emission tomography in large vessel vasculitis. Autoimmun Rev 2019; 18:102405. [PMID: 31648043 DOI: 10.1016/j.autrev.2019.102405] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2019] [Accepted: 06/13/2019] [Indexed: 12/30/2022]
Abstract
With advances in our understanding of the pathogenesis of large vessel vasculitides, we recognise the persistence of inflammation in large vessels, sometimes despite therapy to control clinical symptoms. Achieving an early diagnosis and establishing the extent of disease are important steps in improving our management of these diseases. Imaging is playing an increasing role in the assessment of these patients from diagnosis to prognosis. We review the current and potential role of two important and potentially complementary imaging techniques of magnetic resonance angiography and 18F-fluorodeoxyglucose positron emission tomography in the evaluation of patients with giant cell arteritis and Takayasu arteritis.
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Affiliation(s)
- Daniel Blockmans
- General Internal Medicine, University Hospital Gasthuisberg, Leuven, Belgium
| | - Raashid Luqmani
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science, University of Oxford, Oxford, UK.
| | - Lucia Spaggiari
- Department of Radiology, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
| | - Carlo Salvarani
- Rheumatology Division, Universita' di Modena e Reggio Emilia and Azienda USL-IRCCS di Reggio Emilia, Italy
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Brkic A, Terslev L, Møller Døhn U, Torp‐Pedersen S, Schmidt WA, Diamantopoulos AP. Clinical Applicability of Ultrasound in Systemic Large Vessel Vasculitides. Arthritis Rheumatol 2019; 71:1780-1787. [DOI: 10.1002/art.41039] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2017] [Accepted: 07/09/2019] [Indexed: 12/19/2022]
Affiliation(s)
- Alen Brkic
- Stavanger University Hospital Stavanger Norway
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Predictive value of positive temporal artery biopsies in patients with clinically suspected giant cell arteritis considering temporal artery ultrasound findings. Graefes Arch Clin Exp Ophthalmol 2019; 257:2279-2284. [PMID: 31418104 DOI: 10.1007/s00417-019-04430-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2019] [Revised: 07/08/2019] [Accepted: 07/22/2019] [Indexed: 10/26/2022] Open
Abstract
PURPOSE To investigate the impact of ocular symptom, non-ocular symptom, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and temporal artery ultrasound (TAU) findings on the predictive value of a positive temporal artery biopsy (TAB) in patients with clinically suspected giant cell arteritis (GCA). METHODS In a retrospective, interventional study, data from 68 patients with clinically suspected GCA who underwent TAB between 2015 and 2017 were analysed. Analysis included five parameters: ocular symptom, non-ocular symptom, ESR, CRP level and TAU findings. Using a contingency table, each parameter was separately analysed for the predictive value of a positive TAB, and a discriminant analysis was applied to check for the predictive value of a positive TAB under consideration of all five parameters and of the three strongest predictive parameters. RESULTS A positive TAB was significantly associated with a positive TAU in 15 of 15 patients (p < 0.001), an increased ESR in 37 of 53 patients (p < 0.001), an increased CRP level in 35 of 56 patients (p = 0.004) and non-ocular symptoms in 27 of 40 patients (p = 0.01). A positive TAB was not significantly associated with the presence of ocular symptoms (25 of 46 patients, p = 0.988). Using a discriminant analysis, the combined parameters TAU, ESR and CRP were able to predict a positive TAB in 97.3% of all patients. The positive predictive value was 78.3%, and the negative predictive value was 95.4%. CONCLUSION Temporal artery biopsy to confirm the diagnosis of GCA may not be mandatory in patients who show an elevated ESR and CRP level and a positive TAU.
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Conde-Ferreirós A, Fraile-Alonso MDC, Román-Curto C. "Book Flap" Approach for Temporal Artery Biopsy. Dermatol Surg 2019; 46:1361-1363. [PMID: 31397778 DOI: 10.1097/dss.0000000000002068] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Sundholm JKM, Pettersson T, Paetau A, Albäck A, Sarkola T. Diagnostic performance and utility of very high-resolution ultrasonography in diagnosing giant cell arteritis of the temporal artery. Rheumatol Adv Pract 2019; 3:rkz018. [PMID: 31528841 PMCID: PMC6735832 DOI: 10.1093/rap/rkz018] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/03/2019] [Revised: 07/01/2019] [Indexed: 11/16/2022] Open
Abstract
OBJECTIVE Very-high resolution US (VHRU; 55 MHz) provides improved resolution and could provide non-invasive diagnostic information in GCA of the temporal artery. The objective of this study was to assess the diagnostic utility of VHRU-derived intima thickness (VHRU-IT) in comparison to high-resolution US halo-to-Doppler ratio (HRU-HDR) in patients referred for temporal artery biopsy. METHODS VHRU and HRU of the temporal artery were performed before a biopsy procedure in 78 prospectively recruited consecutive patients who had received glucocorticoid treatment for a median of 8 days (interquartile range 0-13 days) before imaging. Based on the final diagnosis and biopsy findings, the study population was divided into the following four groups: non GCA (n = 40); clinical GCA with no inflammation on biopsy (n = 15); clinical GCA with inflammation limited to adventitia (n = 9); and clinical GCA with transmural inflammation (TMI; n = 11). RESULTS Both VHRU and HRU were useful for identifying subjects with TMI, with VHRU outperforming HRU (area under curve: VHRU-IT 0.99, 95% CI 0.97, 1.00; HRU-HDR 0.74, 95% CI 0.52, 0.96; P=0.026). The diagnostic utility for diagnosing clinical GCA (negative biopsy) or inflammation limited to the adventitia was poor for both VHRU and HRU-HDR. From 5 days after initiation of glucocorticoid treatment, VHRU-IT was increased in eight of nine patients, whereas HRU-HDR was positive in three of seven patients. Both methods showed excellent inter-observer agreement (Cohen's κ: VHRU-IT 0.873; HRU-HDR 0.811). CONCLUSION In suspected GCA, VHRU allows non-invasive real-time imaging of TMI manifestations of the temporal artery wall. VHRU-derived intimal thickness measurement seems to be more sensitive than the halo sign and HRU-HDR in detecting TMI in patients with prolonged glucocorticoid treatment.
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Affiliation(s)
- Johnny K M Sundholm
- Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Tom Pettersson
- Department of General Internal Medicine and Geriatrics, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Anders Paetau
- HUSLAB Meipa1 Pathology Laboratory, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
- Haartman Institute, University of Helsinki, Helsinki, Finland
| | - Anders Albäck
- Department of Vascular Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Taisto Sarkola
- Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
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EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary. Radiol Med 2019; 124:965-972. [PMID: 31254221 DOI: 10.1007/s11547-019-01058-0] [Citation(s) in RCA: 32] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2019] [Accepted: 06/18/2019] [Indexed: 12/25/2022]
Abstract
Large vessel vasculitis (LVV) is the most common form of primary vasculitis comprising of giant cell arteritis (GCA), Takayasu's arteritis (TAK) and idiopathic aortitis. Early diagnosis and treatment of LVV are paramount to reduce the risk of ischemic complications such as visual loss and strokes, vascular stenosis and occlusion, and aortic aneurysm formation. Use of imaging modalities [ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT) and [18F]-fluorodeoxyglucose positron emission tomography (PET)] has steadily increased to enable assessment of cranial and extracranial arteries, as well as the aorta. These imaging modalities are less invasive, more sensitive and readily available compared to temporal artery biopsy (TAB). Modern imaging methods have changed the role of TAB in diagnosing GCA and have replaced diagnostic angiography. Over the last two decades, several studies have evaluated the use of US, MRI, CT and PET in LVV. However, these various imaging tools are not yet uniformly used in routine clinical practice and controversy exists as to which imaging modality best provides meaningful assessments of disease activity and damage in LVV. In January 2018, evidence-based recommendations for the use of imaging modalities in LVV were published. The aim of this review is to summarize the current evidence of imaging in patients with or suspected of having LVV, and to highlight the clinical implications of the EULAR recommendations.
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