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Berling E, Maisonobe T, Morassi O, Echaniz‐Laguna A, Denier C, Le Guillou A, Duschene M, Labeyrie C, Adam C. Factors Distinguishing Nonsystemic Versus Systemic Vasculitic Peripheral Neuropathy: A Retrospective Cohort Study. Eur J Neurol 2025; 32:e70150. [PMID: 40207596 PMCID: PMC11983150 DOI: 10.1111/ene.70150] [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: 11/15/2024] [Revised: 03/17/2025] [Accepted: 03/26/2025] [Indexed: 04/11/2025]
Abstract
BACKGROUND Prompt differentiation of nonsystemic vasculitic neuropathy (NSVN) from systemic vasculitic neuropathy (SVN) without antineutrophil cytoplasmic antibodies or organ involvement (SVN-) is challenging but crucial given the differences in management. Our primary objective was to identify predictors of SVN- versus NSVN. METHODS We retrospectively identified patients seen at either of two centers for histologically definite or probable vasculitic neuropathy diagnosed as SVN- or NSVN. Clinical, electromyographic, laboratory, and histological data at diagnosis and last follow-up were collected. RESULTS We included 98 patients, of whom 46 (47%) had NSVN and 52 (53%) SVN-. Symptoms and neurophysiological findings did not significantly differ between the two groups. By univariate analysis, compared to the NSVN group, the SVN- group had more patients with C-reactive protein ≥ 10 mg/L (44.2% vs. 17.4%, p < 0.01), antinuclear antibodies (42.3% vs. 21.7%, p = 0.04), and low C3 or C4 (23.1% vs. 4.3%, p = 0.02). All three associations were confirmed by multivariate analysis. By multivariate analysis adjusted for symptom duration, muscle vasculitis also was associated with SVN- (OR, 8.33; 95% CI, 2.63-25.00; p < 0.01). CONCLUSION Simple, readily available laboratory and histological variables may help to rapidly distinguish SVN from NSVN.
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Affiliation(s)
- Edouard Berling
- Service de NeurologieAP‐HP, Hôpital Raymond Poincaré, Centre de référence Nord‐Est‐Ile‐de‐France, FHU PHENIXGarchesFrance
- Université de Versailles Saint‐Quentin‐En‐Yvelines, U 1179 INSERMParis‐SaclayFrance
| | - Thierry Maisonobe
- Département de Neurophysiologie CliniqueAP‐HP, Groupe Hospitalier Pitié‐SalpêtrièreParisFrance
- Département de NeuropathologieAP‐HP, Groupe Hospitalier Pitié‐SalpêtrièreParisFrance
| | - Olivier Morassi
- Neurology Department and French National Reference Center for Rare Neuropathies (CERAMIC)Bicêtre University HospitalLe Kremlin‐BicêtreFrance
| | - Andoni Echaniz‐Laguna
- Neurology Department and French National Reference Center for Rare Neuropathies (CERAMIC)Bicêtre University HospitalLe Kremlin‐BicêtreFrance
- INSERM U1195, Paris‐Saclay UniversityLe Kremlin‐BicêtreFrance
| | - Christian Denier
- Stroke Units and Department of Neurology of Hôpital Bicêtre, Le Kremlin Bicêtre, Assistance Publique‐Hôpitaux de ParisParis Saclay UniversityParisFrance
| | | | - Mathilde Duschene
- NeurIT (Neuropathies et Innovations Thérapeutiques) UR 20218, Faculties of Medicine and PharmacyUniversity of LimogesLimogesFrance
- Department of Neurology and Department of Pathology, Reference Center for Rare Peripheral NeuropathiesUniversity Hospital of LimogesLimogesFrance
| | - Céline Labeyrie
- Neurology Department and French National Reference Center for Rare Neuropathies (CERAMIC)Bicêtre University HospitalLe Kremlin‐BicêtreFrance
| | - Clovis Adam
- AP‐HP, Service D'anatomopathologie CliniqueCHU BicêtreLe Kremlin‐BicêtreFrance
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Skeik N, Theeler J, Wang J, Bae A, Cho M, Manunga J. Renal Artery Vasculopathy: Case Study and Literature Review. Angiology 2025:33197251316625. [PMID: 39968596 DOI: 10.1177/00033197251316625] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/20/2025]
Abstract
Non-atherosclerotic renal artery vasculopathies are rare and can be caused by heterogenous group of diseases, some of which has bad prognosis. The literature addressing this rare group of disease is scarce. A single center analysis of all adult patients evaluated for renal artery disease including dissection, aneurysm, stenosis, vasculitis, thrombosis, irregularity, and renal infarct treated at our center, from January 2000 to April 2022. Baseline data collected included demographics, comorbidities, vital signs, laboratory values, pathology, genetic testing, and imaging results/diagnoses. Additionally, treatment modality, follow-up, and symptom relief/recurrence from follow-up visits were collected if available. Out of the included 227 patients, 91 (40%) had renal infarction, 70 (31%) had renal artery dissection, 69 (30%) had aneurysm, 33 (15%) had stenosis, and 32 (14%) had thrombosis. The most common diagnoses were fibromuscular dysplasia (n = 86, 38%) and thromboembolism (n = 38, 17%). Most patients improved with conservative management, reserving endovascular or surgical interventions for symptomatic patients with more complicated presentations. To our knowledge, this is the only case study and general review in the literature that addresses the diagnosis and management of non-atherosclerotic renal arteriopathies showing good outcome for most of the underlying etiologies.
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Affiliation(s)
- Nedaa Skeik
- Section of Vascular and Endovascular Surgery Minneapolis Heart Institute at Abbott Northwestern Hospital Minneapolis, MN, USA
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Johanna Theeler
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Julia Wang
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Aaron Bae
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Mansoo Cho
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Jesse Manunga
- Section of Vascular and Endovascular Surgery Minneapolis Heart Institute at Abbott Northwestern Hospital Minneapolis, MN, USA
- Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
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3
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Zhou C, Zhu W, Zhao J, Shi J, Peng M, Wang C. A 51-Year-Old Man With Dyspnea and a Pulmonary Nodule. Chest 2025; 167:e13-e17. [PMID: 39794082 DOI: 10.1016/j.chest.2024.03.048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2023] [Revised: 03/10/2024] [Accepted: 03/29/2024] [Indexed: 01/13/2025] Open
Abstract
CASE PRESENTATION A 51-year-old man presented with chest tightness, exertional dyspnea, and occasional chest pain for 2 years. The patient visited his local hospital initially, and CT scan revealed a ground glass opacity (GGO) located in the right upper lobe (Fig 1A). He was diagnosed as having pulmonary infection and treated with levofloxacin for 12 days. A repeated chest CT scan 14 days later demonstrated a progressed solid nodule with surrounding ground glass opacity (Fig 1B). With a suspicion of carcinoma in situ, right upper lobectomy was performed via video-assisted thoracoscopic surgery at the local hospital. However, the histologic examination did not show any evidence of malignancy, and the symptoms persisted. Fourteen months later, his dyspnea worsened with extremely low exercise tolerance. The patient denied other symptoms (eg, rash, fever, joint pain, aphthous stomatitis, genital ulceration, other symptoms of arteritis). His appetite was decreased but without significant weight loss. He did not smoke and had a history of fully recovered cerebral infarction 9 months ago. There was no family history of respiratory diseases. After 4 months, a CT pulmonary angiography scan revealed filling defects at the left pulmonary artery and left inferior pulmonary artery (Fig 2A). A vascular narrowing was detected at the left superior pulmonary artery. Accompanied with an increased D-dimer level (> 10 mg/L; normal range, 0-0.5 mg/L), a diagnosis of pulmonary embolism was made. The patient was treated with warfarin, and his symptom of dyspnea was partially relieved. He came to our hospital for further treatment 4 months later.
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Affiliation(s)
- Chunsheng Zhou
- 4+4 Medical Doctor Program, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Wenyan Zhu
- Departments of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jiuliang Zhao
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Juhong Shi
- Departments of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Min Peng
- Departments of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
| | - Chen Wang
- State Key Laboratory of Respiratory Health and Multimorbidity, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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4
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Deniz R, Güzelbey T, Özgür DS, Karaalioğlu B, Akkuzu G, Yıldırım F, Bes C. Isolated inferior thyroidal artery vasculitis: A rare cause of neck pain. Int J Rheum Dis 2023; 26:2294-2296. [PMID: 37191117 DOI: 10.1111/1756-185x.14733] [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: 03/09/2023] [Accepted: 05/05/2023] [Indexed: 05/17/2023]
Abstract
Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non-invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.
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Affiliation(s)
- Rabia Deniz
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Tevfik Güzelbey
- Department of Radiology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Duygu Sevinç Özgür
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Bilgin Karaalioğlu
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Gamze Akkuzu
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Fatih Yıldırım
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Cemal Bes
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
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Ichikawa T, Shimojima Y, Nomura S, Kishida D, Shiozaki M, Tanimura J, Sekijima Y. Testicular vasculitis in eosinophilic granulomatosis with polyangiitis: a case-based review. Clin Rheumatol 2023; 42:293-299. [PMID: 36414864 DOI: 10.1007/s10067-022-06453-z] [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: 10/18/2022] [Revised: 11/10/2022] [Accepted: 11/11/2022] [Indexed: 11/23/2022]
Abstract
Testicular vasculitis (TV) develops when an organ is involved in systemic vasculitis. A 47-year-old man with eosinophilic granulomatosis with polyangiitis (EGPA) developed TV as the first clinical episode. The patient had bronchial asthma for 8 years and developed left testicular pain before developing arthralgia, abdominal involvement, and sensory polyneuropathy, which led to the diagnosis of EGPA. The induration of the affected testicle persisted even after initiating immunosuppressive therapy with corticosteroids and cyclophosphamide, raising concern for testicular neoplasm, while testicular pain and other symptoms resolved. The patient underwent inguinal orchiectomy, and a histology examination of the resected testicle revealed fibrinoid necrotizing vasculitis. Only three cases of biopsy-proven TV in patients with EGPA have been reported in our review of published English-language articles. Two of the three patients in the reviewed cases developed TV before being diagnosed with EGPA. Moreover, all patients underwent extirpation of the affected testicle, leading to a pathological diagnosis of TV. This report suggests that TV may develop and be the presenting condition in EGPA, although urogenital involvement is rare.
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Affiliation(s)
- Takanori Ichikawa
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
| | - Yasuhiro Shimojima
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.
| | - Shun Nomura
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
| | - Dai Kishida
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
| | - Masashi Shiozaki
- Department of Urology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
| | - Jun Tanimura
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
- Department of Neurology, Aizawa Hospital, 2-5-1 Honjo, Matsumoto, 390-8510, Japan
| | - Yoshiki Sekijima
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan
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Skeik N, Smith J, Olson SL, Lohese OL, Mirza A, Manunga J. Mesenteric Artery Dissection and Wall-Thickening, Case Study and General Review. Angiology 2022:33197221100601. [PMID: 35921630 DOI: 10.1177/00033197221100601] [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] [Indexed: 11/16/2022]
Abstract
Mesenteric artery dissection (D) and wall-thickening (WT) are rare vasculopathies that can lead to serious complications. This is a single center analysis of all patients evaluated for mesenteric arterial (celiac, superior (SMA) and/or inferior mesenteric (IMA)) D and/or WT from January 1, 2000, to January 31, 2020 at our hospital. Among the 101 included patients, the average age was 55.6 ± 13.6 years, mostly affecting men (62%). There were 20 celiac artery D, 8 WT, 15 D with WT, 15 SMA D, 7 WT, 8 D with WT, one IMA D, two WT, and 25 with multiple arterial involvement. Primary etiologies included segmental arterial mediolysis (SAM) (n = 17), isolated D (n = 17), localized vasculitis of the gastrointestinal tract (LVGT) (n = 16), fibromuscular dysplasia (FMD) (n = 13), extension of thoracoabdominal aortic D (n = 12), and trauma (n = 12). Most (71%) patients presented with abdominal pain. Hypertension (55%), hyperlipidemia (33%) and tobacco use (31%) were prevalent. Management included conservative (22%), medical (47%), endovascular (19%), and/or open repair (12%) with high in-hospital survival (98%) and symptom relief (73%). Our paper complements the scarce literature addressing the diagnosis and management of rare mesenteric vasculopathies. Most patients improved with conservative management, reserving endovascular or surgical interventions for symptomatic patients with more complicated presentations.
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Affiliation(s)
- Nedaa Skeik
- Section of Vascular and Endovascular Surgery, Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, USA.,51432Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Jenna Smith
- 51432Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Sydney L Olson
- 12244Northwestern University Feiberg School of Medicine, Chicago, IL, USA
| | - Opema L Lohese
- 51432Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
| | - Aleem Mirza
- Department of Cardiovascular and Vascular Surgery, 12340University of Texas Health Science, Houston, TX, USA
| | - Jesse Manunga
- Section of Vascular and Endovascular Surgery, Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, USA.,51432Minneapolis Heart Institute Foundation, Minneapolis, MN, USA
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Martins-Martinho J, Dourado E, Khmelinskii N, Espinosa P, Ponte C. Localized Forms of Vasculitis. Curr Rheumatol Rep 2021; 23:49. [PMID: 34196889 PMCID: PMC8247627 DOI: 10.1007/s11926-021-01012-y] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/21/2021] [Indexed: 12/26/2022]
Abstract
Purpose of Review To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis. Recent Findings Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Summary Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted.
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Affiliation(s)
- Joana Martins-Martinho
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal. .,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
| | - Eduardo Dourado
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Nikita Khmelinskii
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Pablo Espinosa
- Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal
| | - Cristina Ponte
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal
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An uncommon form of localized colonic eosinophilic vasculitis with extensive thrombosis of the spleen and liver: A case report and literature review. Int J Surg Case Rep 2020; 75:50-52. [PMID: 32919329 PMCID: PMC7490986 DOI: 10.1016/j.ijscr.2020.09.006] [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: 08/04/2020] [Revised: 09/01/2020] [Accepted: 09/02/2020] [Indexed: 11/21/2022] Open
Abstract
INTRODUCTION Localized vasculitis of the gastrointestinal tract is an uncommon disease that mainly presents as polyarteritis nodosa and is mainly located on small bowel and gall bladder. Localized eosinophilic vasculitis of the colon, which needs surgical intervention, has never been reported before. CASE PRESENTATION A 40-year-old man was diagnosed with localized eosinophilic vasculitis of the colon with an initial presentation of necrotizing colitis of ascending colon. After right hemicolectomy, extensive thrombosis of the liver and spleen occurred with the presentation of abdominal pain. The histopathological analysis revealed ischemic colitis with eosinophilic vasculitis in medium-sized vessels throughout the colon. The thrombosis was improved after prednisolone and azathioprine were given. The results of all autoimmune tests, including those for anti-neutrophil cytoplasmic antibodies, were all negative except the elevation of serum immunoglobulin E (680 kU/L [normal, <25 kU/L]). CONCLUSION Although the patient failed to meet the criteria of the Churg-Strauss syndrome, this case may represent an atypical localized variant of eosinophilic vasculitis of the gastrointestinal tract. Immunosuppressant therapy should be considered after surgery.
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9
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Bains HK, Agostinho N. Rare cause of bowel ischaemia: localized vasculitis of the gastrointestinal tract. ANZ J Surg 2019; 90:1520-1522. [PMID: 31833190 DOI: 10.1111/ans.15625] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2019] [Revised: 11/20/2019] [Accepted: 11/22/2019] [Indexed: 11/30/2022]
Affiliation(s)
- Harinder K Bains
- Department of Colorectal Surgery, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
| | - Nelson Agostinho
- Department of Colorectal Surgery, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
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Skeik N, Olson SL, Hari G, Pavia ML. Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases. Vasc Med 2019; 24:549-563. [DOI: 10.1177/1358863x19873410] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Segmental arterial mediolysis (SAM) is a rare but serious nonatherosclerotic, noninflammatory vasculopathy of unknown etiology that often results in dissection, aneurysm, occlusion, or stenosis of, primarily, the abdominal arteries. Current literature lacks consensus on diagnostic criteria and management options for SAM. This review summarizes 143 cases and aims to advance appropriate recognition and management of SAM. Literature review of all relevant SAM case studies from 2005 to 2018 yielded 126 individual SAM cases from 66 reports. We identified 17 additional SAM cases from our center, bringing our analysis to 143 patients. Patients with SAM were most commonly men (68%) in their 60s. Hypertension (43%), tobacco use (12%), and hyperlipidemia (12%) were common comorbidities. Abdominal pain (80%) and intraabdominal bleeding (50%) were the most common presenting symptoms. Computed tomography was the most frequently used imaging method (78%), and histology was available in 44% of cases. The most commonly affected vessels were the superior mesenteric (53%), hepatic (45%), celiac (36%), renal (26%), and splenic (25%) arteries with aneurysm (76%), dissection (61%), and arterial rupture (46%). Treatments included coil embolization (28%), abdominal organ surgery (24%), open arterial repair (21%), and medical management (20%). Case-specific treatment modalities yielded symptom relief in the vast majority (91%) of patients, with a mortality rate of 7%.
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Affiliation(s)
- Nedaa Skeik
- Vascular Medicine Section, Minneapolis Heart Institute, Minneapolis, MN, USA
| | - Sydney L Olson
- Vascular Medicine Section, Minneapolis Heart Institute, Minneapolis, MN, USA
| | - Gopika Hari
- Vascular Medicine Section, Minneapolis Heart Institute, Minneapolis, MN, USA
| | - Mary L Pavia
- Vascular Medicine Section, Minneapolis Heart Institute, Minneapolis, MN, USA
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11
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Skeik N, Hyde JR, Olson SL, Thaler CM, Abuatiyeh W, Ahmed AK, Lyon DR, Witt DR, Garberich R, Sullivan T. Nonatherosclerotic Abdominal Vasculopathies. Ann Vasc Surg 2019; 60:128-146. [DOI: 10.1016/j.avsg.2019.04.004] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/17/2018] [Revised: 03/11/2019] [Accepted: 04/04/2019] [Indexed: 11/28/2022]
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12
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An Autopsy Case of a 5-Year-Old Child with Acute Pancreatitis Caused by Eosinophilic Granulomatosis with Polyangiitis-like Necrotizing Vasculitis. Case Rep Rheumatol 2019; 2019:9053747. [PMID: 31565459 PMCID: PMC6745468 DOI: 10.1155/2019/9053747] [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: 05/25/2019] [Accepted: 08/08/2019] [Indexed: 11/18/2022] Open
Abstract
In children, acute pancreatitis has been reported in IgA vasculitis, Kawasaki disease, systemic lupus erythematosus-associated vasculitis, and juvenile dermatomyositis-associated vasculitis. However, its frequency in these vasculitides has been shown to be low. In other childhood-onset vasculitides, acute pancreatitis is seldom reported. The patient was a 5-year-old Japanese boy who suddenly presented with gastrointestinal (GI) bleeding. Therapy with antiulcer drugs successfully stopped bleeding, but subsequently, high fever, leukocytosis, and hypoxia appeared. He died 12 days after he presented with GI bleeding. An autopsy unexpectedly revealed that necrotizing vasculitis with marked eosinophilic and histiocytic infiltration of the pancreas led to acute pancreatitis, and gastric ulcer with eosinophilic infiltration was shown to be the origin of GI bleeding. In addition, eosinophilic infiltration was found in the small intestine, lungs, and bone marrow. Necrotizing vasculitis with eosinophilic and histiocytic infiltration of the pancreas, eosinophilic infiltration of the airway wall, and eosinophilic gastroenteritis with gastric ulcer were histologically confirmed, suggesting that the present case may be an early stage of eosinophilic granulomatosis with polyangiitis- (EGPA-) like vasculitis. To our knowledge, this might be the first reported case of EGPA-like vasculitis presenting with acute pancreatitis in a child.
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13
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Altabás-González I, Pérez-Gómez N, Pego-Reigosa JM. How to investigate: Suspected systemic rheumatic diseases in patients presenting with muscle complaints. Best Pract Res Clin Rheumatol 2019; 33:101437. [PMID: 31810549 DOI: 10.1016/j.berh.2019.101437] [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] [Indexed: 01/20/2023]
Abstract
Muscular symptoms, which may be due to multiple causes, are one of the most common early complaints in a rheumatology practice. Musculoskeletal symptoms in rheumatic conditions are very varied, ranging from mechanical problems to muscular symptoms derived from inflammatory and systemic autoimmune diseases. Several drugs commonly used by different specialists and certain drugs used in rheumatology can also cause a wide variety of muscle symptoms. A description of different systemic autoimmune diseases follows to describe the different forms of involvement of the musculoskeletal system that they cause, as well as the main causes with which a differential diagnosis should be made. In this chapter, we will try to give some clues to reach an early diagnosis using clinical criteria, particularly based on a directed anamnesis and physical examination, discussing possible guidelines for the complimentary tests that may be required in patients with muscle complaints.
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Affiliation(s)
- Irene Altabás-González
- Rheumatology Department, University Hospital of Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) Study Group, Health Research Institute from Galicia Sur (IISGS), Consulta n. 4 (Planta 0), Alto do Meixoeiro s/n, 36214, Vigo, Spain.
| | - Naír Pérez-Gómez
- Rheumatology Department, University Hospital of Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) Study Group, Health Research Institute from Galicia Sur (IISGS), Consulta n. 4 (Planta 0), Alto do Meixoeiro s/n, 36214, Vigo, Spain.
| | - José María Pego-Reigosa
- Rheumatology Department, University Hospital of Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases) Study Group, Health Research Institute from Galicia Sur (IISGS), Consulta n. 4 (Planta 0), Alto do Meixoeiro s/n, 36214, Vigo, Spain.
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14
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Efficacy and safety of Rituximab in vasculitic neuropathy: a systematic review of the literature. ACTA ACUST UNITED AC 2019; 15:173-178. [PMID: 30691946 DOI: 10.1016/j.reuma.2018.10.007] [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: 05/23/2018] [Revised: 09/25/2018] [Accepted: 10/18/2018] [Indexed: 12/25/2022]
Abstract
OBJECTIVE To review the efficacy and safety of rituximab in vasculitic neuropathy (VN) METHODS: A literature search was performed on Medline and Embase up until 2017. It included terms related to "vasculitis","vasculitic neuropathy" and "Rituximab". Research was carried out by two reviewers. The main outcome was rituximab efficacy. RESULTS Of an initial selection of 702 articles, 5 remained with a level of evidence between 1+ and 3 and variable recommendation degree. In the only clinical trial included, rituximab was superior to conventional therapy for cryoglobulinemic vasculitis with VN showing an increase in drug retention rate (64.3% vs. 3.5%; P<.001)and with a lower rate of serious adverse effects (.12 vs. .48). Cohort studies of patients with cryoglobulinemic vasculitis showed improvement and complete/partial remission of VN. In a series of 5 cases of refractory EGPA suffering from VN, 60% and 20% of patients achieved complete and partial remission respectively. CONCLUSIONS Rituximab seems an effective and safe treatment for VN in the context of cryoglobulinemic vasculitis. Evidence for specific efficacy in VN in the context of other types of vasculitis is lacking.
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Pulmonary Artery Compression and Invasion by a Ruptured Giant Thoracic Aortic Aneurysm: A Rare Presentation. ACTA ACUST UNITED AC 2018; 2:201-206. [PMID: 30370383 PMCID: PMC6200667 DOI: 10.1016/j.case.2018.02.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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Koster MJ, Warrington KJ. Vasculitis of the mesenteric circulation. Best Pract Res Clin Gastroenterol 2017; 31:85-96. [PMID: 28395792 DOI: 10.1016/j.bpg.2016.12.003] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/30/2016] [Revised: 12/09/2016] [Accepted: 12/17/2016] [Indexed: 01/31/2023]
Abstract
Vasculitis of the mesenteric circulation is an uncommon but life-threatening manifestation of systemic vasculitis. Initial symptoms are frequently non-specific and therefore patients often present to primary care physicians and gastroenterologists with abdominal pain or gastrointestinal bleeding. Given the severity of the conditions associated with mesenteric vasculitis, it is imperative to appropriately diagnose and initiate treatment of suspected cases. This review will focus on diseases commonly associated with vasculitis of the mesenteric vessels. Imaging characteristics and clinical features assisting in diagnosis as well as initial approaches to treatment are emphasized.
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Affiliation(s)
- Matthew J Koster
- Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA.
| | - Kenneth J Warrington
- Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA.
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Testicular Vasculitis: A Sonographic and Pathologic Diagnosis. Case Rep Radiol 2017; 2017:8923621. [PMID: 28246567 PMCID: PMC5299182 DOI: 10.1155/2017/8923621] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2016] [Revised: 12/19/2016] [Accepted: 01/10/2017] [Indexed: 11/17/2022] Open
Abstract
Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject.
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Gözüküçük M, Gürsoy AY, Kankaya D, Atabekoglu C. Single-organ vasculitis of the cervix accompanying human papillomavirus infection. Interv Med Appl Sci 2016; 8:93-95. [PMID: 28386466 DOI: 10.1556/1646.8.2016.2.111] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
Single-organ vasculitis (SOV) has rarely been reported to involve the female genital tract but mostly the uterine cervix. A 39-year-old woman was diagnosed to have a high-grade cervical intraepithelial lesion and was treated by large loop excision of the transformation zone. Histopathological evaluation of the excised specimen confirmed the diagnosis of cervical intraepithelial neoplasia grade III accompanied by human papillomavirus infection. The excised second specimen showed the evidence of vasculitis of medium-sized vessels of the cervix, which is a quite rare form of SOV. It seems to be important to be aware of the localized form of polyarteritis nodosa limited to the female genital tract to prevent unnecessary immunosuppressive therapies.
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Affiliation(s)
- Murat Gözüküçük
- Department of Obstetrics and Gynecology, Kayseri Training and Research Hospital , Kayseri, Turkey
| | - Aslı Yarcı Gürsoy
- Department of Obstetrics and Gynecology, Ufuk University Medical Faculty , Ankara, Turkey
| | - Duygu Kankaya
- Department of Pathology, Ankara University Medical Faculty , Ankara, Turkey
| | - Cem Atabekoglu
- Department of Obstetrics and Gynecology, Ankara University Medical Faculty , Ankara, Turkey
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Taniguchi Y, Ogata K, Yoshimatsu R, Yamagami T, Terada Y. Polyarteritis Nodosa of the Iliopsoas Muscle. Arthritis Rheumatol 2015; 67:2426. [DOI: 10.1002/art.39206] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2015] [Accepted: 05/14/2015] [Indexed: 11/05/2022]
Affiliation(s)
| | - Koji Ogata
- Kochi Medical School; Nankoku, Kochi Japan
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Isolated pulmonary vasculitis: Case report and literature review. Semin Arthritis Rheum 2015; 44:514-517. [DOI: 10.1016/j.semarthrit.2014.10.003] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2014] [Revised: 09/30/2014] [Accepted: 10/10/2014] [Indexed: 11/21/2022]
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21
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Vaskulitiden. Herz 2015; 40:85-98. [DOI: 10.1007/s00059-014-4200-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
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Sharma A, Gopalakrishan D, Nada R, Kumar S, Dogra S, Aggarwal MM, Gupta R, Minz RW, Kakkar N, Vashishtha RK, Singh S. Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades. Int J Rheum Dis 2014; 17:562-572. [PMID: 24237487 DOI: 10.1111/1756-185x.12223] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
INTRODUCTION Systemic vasculitides are great masqueraders and at times their presenting manifestations can be very different from the usual recognized patterns. Such uncommon presentations of granulomatosis with polyangiitis (Wegener's granulomatosis), classical polyarteritis nodosa and unclassifiable vasculitides are described here with the relevant review of literature. METHODS All patients diagnosed as having systemic vasculitides and classified as having granulomatosis with polyangiitis (Wegener's granulomatosis), classic polyarteritis nodosa, microscopic polyangiitis and unclassifiable vasculitis according to EMEA consensus methodology and followed up prospectively from June 2007 to December, 2011 were included. Details of uncommon presentations of these disorders were identified. RESULTS Seventy-nine patients with systemic vasculitides were seen under our rheumatology services during this period. These included 45 patients with granulomatosis with polyangiitis (Wegener's granulomatosis), 18 with classic polyarteritis nodosa, five with microscopic polyangiitis, four with Churg-Strauss syndrome and seven with unclassifiable vasculitis. The uncommon presentations of granulomatosis with polyangiitis were a tumefactive subcutaneous mass in the thigh; prostatomegaly with obstructive uropathy and advanced renal failure; and predominant gastrointestinal (GI) vasculitis with thrombocytopenia and coagulopathy at presentation. The uncommon manifestations of classic polyarteritis nodosa were secondary antiphospholipid antibody syndrome and Budd-Chiari syndrome. One patient with massive lower GI bleeding required surgical resection of the large bowel which showed isolated necrotizing granulomatous GI vasculitis. Single organ vasculitis of the GI tract was diagnosed. CONCLUSIONS Systemic necrotizing vasculitides may present with uncommon manifestations and a high index of suspicion is required for early diagnosis and prompt treatment to prevent adverse outcomes.
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Affiliation(s)
- Aman Sharma
- Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Large vessel vasculitis in elderly patients: early diagnosis and steroid-response evaluation with FDG-PET/CT and contrast-enhanced CT. Rheumatol Int 2014; 34:1545-54. [PMID: 24643395 DOI: 10.1007/s00296-014-2985-3] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2014] [Accepted: 03/04/2014] [Indexed: 12/16/2022]
Abstract
Large vessel vasculitis (LVV) is an often-reported cause of inflammation of unknown origin (IUO) in elderly people. The objective of this study was to describe the usefulness of fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and contrast-enhanced CT in early diagnosis and treatment follow-up of patients with LVV presenting as elderly onset IUO. We retrospectively compared contrast-enhanced CT findings and FDG-PET/CT findings of the patients diagnosed with LVV and 11 controls; all subjects were 50 years of age or older. We evaluated maximum standardised uptake value (SUV(max)) and PET score of the aortic wall for quantitative comparison of FDG-PET/CT findings. We measured the aortic wall thickness (W) and its ratio against the radius (W/R) for quantitative comparison of aortic wall thickening by contrast-enhanced CT. After steroid treatment, we compared these values with those pre-treatment. Of 124 patients who were hospitalised due to advanced age and IUO, 88 underwent FDG-PET/CT and contrast-enhanced CT. Abnormal findings were observed on images from 78 patients. The findings were indicative of LVV in 13 patients (10.5 %), of whom more than half had only non-specific symptoms. Patients with LVV had significantly higher aortic wall SUV(max) (3.85 vs. 1.95), PET scores by FDG-PET/CT, and aortic wall thicknesses by contrast-enhanced CT (3.8 vs. 2.6 mm) than controls. Significant improvement in aortic wall thickening was evidenced by reduced PET scores and by contrast-enhanced CT findings in patients who were followed up after treatment. LVV is an important cause of IUO with non-specific symptoms in elderly patients. Imaging examination comprising contrast-enhanced CT and FDG-PET/CT is useful for early diagnosis and early treatment evaluation of LVV, allowing for amelioration of reversible aortic wall thickening.
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Song ST, Kim Y, Park CK, Yoo SJ, Kim JH, Kang SW, Yoo IS. Localized Mesenteric Vasculitis in a Patient with Polymyalgia Rheumatica. JOURNAL OF RHEUMATIC DISEASES 2014. [DOI: 10.4078/jrd.2014.21.5.253] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Seung Taek Song
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Young Kim
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Chan Keol Park
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Su Jin Yoo
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Jin Hyun Kim
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Seong Wook Kang
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - In Seol Yoo
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
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de Souza AWS, Pereira Rosa D, Pirozzi Buosi AL, Oliveira ACD, Natour J. Vasculite testicular – uma manifestação rara de artrite reumatoide. REVISTA BRASILEIRA DE REUMATOLOGIA 2013. [DOI: 10.1590/s0482-50042013000400009] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
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Atisha-Fregoso Y, Hinojosa-Azaola A, Alcocer-Varela J. Localized, single-organ vasculitis: clinical presentation and management. Clin Rheumatol 2012; 32:1-6. [PMID: 22918493 DOI: 10.1007/s10067-012-2069-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2012] [Accepted: 08/09/2012] [Indexed: 10/28/2022]
Abstract
The term vasculitis usually evokes a systemic disease with catastrophic outcomes; however, vasculitides may also present in a localized form, with a better prognosis when compared with their systemic counterpart. In order to avoid confusion and facilitate classification, the term single-organ vasculitis (SOV) has been proposed. Remarkably, current criteria for the classification of the vasculitis do not include the SOV term, due in part to the lack of appropriate definitions, since most data come from case series; moreover, the scarce information available is also extremely heterogeneous. This review focuses on the epidemiology, clinical course, prognosis, and suggested treatment of the SOV, with emphasis in the most recent information available.
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Affiliation(s)
- Yemil Atisha-Fregoso
- Departamento de Inmunología y Reumatología, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15 Col. Sección XVI, Tlalpan, 14080, México, D.F., México
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Hernández-Rodríguez J, Tan CD, Koening CL, Khasnis A, Rodríguez ER, Hoffman GS. Testicular vasculitis: findings differentiating isolated disease from systemic disease in 72 patients. Medicine (Baltimore) 2012; 91:75-85. [PMID: 22391469 DOI: 10.1097/md.0b013e31824156a7] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Testicular vasculitis (TV) may be part of systemic (testicular) vasculitis (STV) or may exist as single-organ/isolated (testicular) vasculitis (ITV). In the current study we sought to identify clinical and histologic features that distinguish STV from ITV. The distinction was deemed important because it is already well established that in other forms of single organ vasculitis, surgical therapy alone may be curative. We identified patients with biopsy-proven TV from pathology databases from our institution and from an English-language PubMed search. Patients were included if data were available to determine TV extent confidently. Data recorded included clinical, laboratory, and histologic features; treatment; and clinical follow-up. The study included 72 patients with TV (mean age, 42 yr; range, 4-78 yr) (7 from our institution). About 74% of patients presented with painful testicular swelling/mass, 10% with a painless testicular swelling/mass, and 4% with epididymal swelling/mass. Eleven percent had no testicular complaints and vasculitis was discovered at autopsy or in other surgical interventions. Vasculitis involved the testicle in 80.3% of cases, the epididymis in 44.6%, and the spermatic cord in 30.6%. Thirty-seven (51%) patients had ITV and 35 (49%) had STV. No differences between ITV and STV patients were found in regards to age, presenting testicular features, duration of testicular symptoms, and time of follow-up. Compared to ITV patients, STV patients presented more often with constitutional/musculoskeletal symptoms (74.3% vs. 8.3%, respectively; p = 0.0001), elevated erythrocyte sedimentation rate (94.7% vs. 16%; p = 0.0001), and anemia (50% vs. 0%; p = 0.0001). Neoplasm was more frequently suspected in ITV than in STV (74.2% vs. 31.6%; p = 0.001), but only occurred in 2 ITV patients. Long-term glucocorticoid therapy was given only to STV patients, and 59.1% of them also received cytotoxic agents. ITV was diagnosed more often by orchiectomy (81.1% vs. 42.9%; p = 0.001) and less frequently by testicular biopsy (2.7% vs. 28.6%; p = 0.003) than STV. Nongranulomatous inflammation affecting medium-sized vessels occurred in most patients with both ITV and STV. Among STV, polyarteritis nodosa was the most frequently diagnosed (63%), followed by Wegener granulomatosis (17%).In summary, TV occurs as ITV in men usually presenting with a testicular mass in the absence of systemic symptoms and normal laboratory results. In most ITV patients, a testicular neoplasm is initially suspected, and TV is an unexpected finding. After surgical removal, ITV does not require systemic therapy. Polyarteritis nodosa is the systemic vasculitis most frequently associated with testicular involvement.
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Affiliation(s)
- José Hernández-Rodríguez
- From the Department of Autoimmune and Systemic Diseases, Hospital Clínic, Barcelona, Spain (JHR); Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases (AK, GSH) and Department of Anatomic Pathology (CDT, ERR), Cleveland Clinic, Cleveland, Ohio; and Division of Rheumatology (CLK), University of Utah, Salt Lake City, Utah
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Benz N, Daikeler T, Frank S, Mehling M, Tyndall A, Trendelenburg M. Three cases of primary small vessel vasculitis of the skeletal muscle-an own entity. BMJ Case Rep 2011; 2011:bcr.08.2011.4631. [PMID: 22674106 DOI: 10.1136/bcr.08.2011.4631] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Whereas systemic vasculitis is the most common form of vasculitis, vasculitis restricted to a single organ system is rare. Primary vasculitis restricted to striated skeletal muscle has been described in few case reports for polyarteritis nodosa and leucocytoclastic vasculitis, but not for small vessel vasculitis, type microscopic polyangiitis. The authors describe three patients with primary small vessel vasculitis of the skeletal muscle without evidence of other major organ involvement. All three patients presented with myalgias and highly elevated acute phase reactants while muscle weakness and elevated creatine kinase levels were not consistently present. Diagnoses were established by muscle biopsy and extensive search for potential causes of secondary vasculitis. Complete remission could be accomplished by steroids alone in only one case, while additional immunosuppressants were needed in the other two cases. Primary small vessel vasculitis of the skeletal muscle should be considered in patients presenting with myalgia and signs of systemic inflammation in the absence of other organ manifestations. Once diagnosed, aggressive systemic immunosuppression is appropriate.
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Affiliation(s)
- Nadja Benz
- Clinic for Internal Medicine, University Hospital, Basel, Switzerland.
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Kim M, Lee HJ, Yeo MK, Lee YS, Moon HS, Lee SI, Cho JS, Song KS. Lymphocytic Phlebitis of the Stomach - A Case Report with Literature Review -. KOREAN JOURNAL OF PATHOLOGY 2011. [DOI: 10.4132/koreanjpathol.2011.45.6.654] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Meeran Kim
- Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea
| | - Hyun-Jung Lee
- Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea
| | - Min-Kyung Yeo
- Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea
| | - Young-Suk Lee
- Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea
| | - Hee Seok Moon
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Sang-Il Lee
- Department of Surgery, Chungnam National University School of Medicine, Daejeon, Korea
| | - June-Sik Cho
- Department of Radiology, Chungnam National University School of Medicine, Daejeon, Korea
| | - Kyu-Sang Song
- Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea
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Abstract
Inflammatory diseases of blood vessels can occur in any part of the vascular system with a variety of clinical and histopathological manifestations. Possible etiologies include primary systemic vasculitis, secondary vasculitis or isolated single organ vasculitis. Key criteria of morphological vasculitis work-up are vessel size, type of inflammation (granulomatous, necrotizing and/or leukocytoclastic) as well as presence or absence of immune complexes and extravascular inflammatory changes. Together with the typical organ involvement and serological data, these criteria constitute the basis of vasculitis classification. A histopathologically confirmed biopsy is the gold standard for a diagnosis of vasculitis, although a definite diagnosis can frequently not be made solely on histopathological grounds. Differential diagnostic overlaps and possible ways of discrimination are presented. In many cases, however, histopathology can only provide a definite diagnosis in combination with clinical and serological data. A conclusive morphological diagnosis depends on the right time of biopsy and selection of appropriate biopsy material.
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Affiliation(s)
- K Holl-Ulrich
- Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck, Germany.
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Salvarani C, Calamia KT, Crowson CS, Miller DV, Broadwell AW, Hunder GG, Matteson EL, Warrington KJ. Localized vasculitis of the gastrointestinal tract: a case series. Rheumatology (Oxford) 2010; 49:1326-35. [DOI: 10.1093/rheumatology/keq093] [Citation(s) in RCA: 47] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
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Manifestations digestives et ischémie mésentérique au cours des vascularites systémiques. ACTA ACUST UNITED AC 2010. [DOI: 10.1007/s11725-010-0201-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
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Holl-Ulrich K, Noack F, Feller AC. [Vasculitis: histopathology and differential diagnosis]. Z Rheumatol 2009; 68:320-8. [PMID: 19337743 DOI: 10.1007/s00393-008-0402-6] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Abstract
A histopathologically confirmed biopsy is the gold standard for the diagnosis of vasculitis. Possible etiologies include primary systemic vasculitis, secondary vasculitis or isolated single organ vasculitis, although on histopathological grounds alone a clear differentiation is frequently not possible. The key criteria of morphological vasculitis work-up include vessel size, type of inflammation (granulomatous, necrotizing and/or leukocytoclastic) as well as the presence or absence of immune complexes and extravascular inflammatory changes. Together with the typical organ involvement and serological data, these criteria constitute the basis of vasculitis classification. Differential diagnostic overlaps and possible discrimination methods are presented. In the same way that the clinical approach of vasculitis patients is an interdisciplinary one, histopathology can only provide a definite diagnosis in combination with clinical and serological data. A conclusive morphological diagnosis depends on the right time of biopsy and the selection of appropriate biopsy material.
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Affiliation(s)
- K Holl-Ulrich
- Referenzzentrum für Vaskulitis-Diagnostik, Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck, Germany.
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LIANG KIMBERLYP, CHOWDHARY VAIDEHIR, MICHET CLEMENTJ, MILLER DYLANV, SUNDT THORALFM, CONNOLLY HEIDIM, CROWSON CYNTHIAS, MATTESON ERICL, WARRINGTON KENNETHJ. Noninfectious Ascending Aortitis: A Case Series of 64 Patients. J Rheumatol 2009; 36:2290-7. [DOI: 10.3899/jrheum.090081] [Citation(s) in RCA: 66] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
Objective.To identify the clinical presentation and histopathologic characteristics of noninfectious ascending aortitis.Methods.A retrospective medical record and histopathology review was performed of patients with histologic evidence of active noninfectious aortitis who underwent ascending aortic aneurysm resection at Mayo Clinic between January 1, 2000, and February 28, 2006. Clinicopathologic features were recorded, including demographics, clinical presentation, laboratory, imaging findings, histopathology, complications, treatment, and outcome.Results.Sixty-four patients (50% women) were identified; the majority were Caucasian (83%) and elderly (mean age 69.1 yrs). Upon initial presentation, 45% had aneurysm-related symptoms, 33% were asymptomatic, 12.5% had constitutional symptoms, 4.7% had symptoms referable to cranial arteries, and 9.4% had polymyalgia rheumatica (PMR) symptoms. The majority (81%) were of “isolated” variant, with no rheumatologic history. Mean preoperative erythrocyte sedimentation rate was 16.2 ± 23.3 mm/h (n = 20). Additional vascular imaging abnormalities were present in 72% of patients, including stenoses and/or ectasia of major aortic branches and descending thoracic or abdominal aneurysms. Giant cells were seen in 71.9%. Median followup time was 15.4 months, during which 6 (9.4%) patients died. Only 22 (34%) patients received corticosteroids, with uncertain effect on development of recurrent aneurysms, rupture, or dissections.Conclusion.Noninfectious ascending aortitis frequently occurs even in the absence of history, symptoms, or signs of giant cell arteritis (GCA) or PMR. When discovered, such patients should be followed closely, as a majority have additional vascular abnormalities. More studies are needed to determine optimal strategies for surveillance, detection, and treatment of ascending aortitis, which may represent a clinical entity distinct from classical GCA.
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Hernández-Rodríguez J, Tan CD, Rodríguez ER, Hoffman GS. Gynecologic vasculitis: an analysis of 163 patients. Medicine (Baltimore) 2009; 88:169-181. [PMID: 19440120 DOI: 10.1097/md.0b013e3181a577f3] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023] Open
Abstract
Gynecologic vasculitis (GynV) has been reported as part of systemic vasculitis (SGynV) and as single-organ (isolated gynecologic) vasculitis (IGynV). In the current study, we analyzed the clinical and histologic characteristics of patients with GynV and sought to identify features that differentiate the isolated from the systemic forms of the disease. We used pathology databases from our institution and an English-language literature search (PubMed) to identify affected patients with biopsy-proven GynV. Using a standardized format for data gathering and analysis, we recorded clinical manifestations, laboratory and histologic features, and surgical and medical therapies. Patients were analyzed as 2 subsets: IGynV and SGynV.A total of 163 patients with GynV were included (152 from the literature and 11 from the Cleveland Clinic pathology database). The incidence of vasculitis among all gynecologic surgeries in our institution over 16 years was 0.15%. Half of the patients presented with vaginal bleeding. Other less common presentations included the finding of an asymptomatic abdominal mass, uterine prolapse, atypical cervical smear, and pelvic pain. Constitutional and musculoskeletal symptoms were reported in 24% of patients. One hundred fifteen (70.6%) patients had IGynV, and 48 (29.4%) had SGynV. Compared to patients with SGynV, those with IGynV were younger (median age, 51 yr; range, 18-80 yr vs. median, 68 yr; range, 32-83 yr; p = 0.0001) and presented more often with vaginal bleeding (57% vs. 25%; p = 0.0002) and less frequently with asymptomatic pelvic masses (6% vs. 35%; p = 0.0001). IGynV was less often associated with constitutional or musculoskeletal symptoms (7% vs. 74%; p = 0.0001). Patients with IGynV were much less likely to have abnormal erythrocyte sedimentation rates (26% vs. 97%; p = 0.0001) and anemia (17% vs. 80%; p = 0.0001) than patients with SGynV. None of the patients with IGynV received corticosteroids, whereas almost all patients with SGynV received corticosteroids and about one-third also received cytotoxic therapy. In IGynV, the site most often involved was the uterus, particularly the cervix, whereas in SGynV lesions were more often multifocal, affecting mainly ovaries, fallopian tubes, and myometrium. Nongranulomatous inflammation occurred in most patients with IGynV, while the predominant histologic pattern noted in SGynV was granulomatous.While vasculitis was the only lesion in 32% of the resected specimens, leiomyomas (18.4%) and endometrial carcinoma (8.3%) were the most frequent concomitant benign and malignant (nonvasculitic) lesions, respectively. Except for benign ovarian abnormalities, which were more frequent in SGynV than in IGynV (21% vs. 4%; p = 0.001), other benign (50%) and malignant (18%) conditions were similarly present in both groups. Among SGynV patients, giant cell arteritis was diagnosed in 29 of the 48 (60.4%) patients, and one-third presented without symptoms of vascular involvement or polymyalgia rheumatica. In summary, GynV is rare and most often occurs as a single-organ disease. It is usually an incidental finding in the course of surgery. The isolated form is associated with the absence of systemic symptoms and normal acute phase reactants, and does not require systemic therapy. Among systemic vasculitides, giant cell arteritis is the most frequently reported form of systemic vasculitis with gynecologic involvement.
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Affiliation(s)
- José Hernández-Rodríguez
- From the Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases (JHR, GSH) and Department of Anatomic Pathology (CDT, ERR), Cleveland Clinic, Cleveland, Ohio
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Hernández-Rodríguez J, Tan CD, Molloy ES, Khasnis A, Rodríguez ER, Hoffman GS. Vasculitis involving the breast: a clinical and histopathologic analysis of 34 patients. Medicine (Baltimore) 2008; 87:61-69. [PMID: 18344804 DOI: 10.1097/md.0b013e31816a8d1f] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Vasculitis of the breast (VB) may be an isolated finding or a manifestation of systemic vasculitis. In the current study we sought to characterize isolated VB (IVB) and compare it to VB in the setting of systemic vasculitis. We studied VB cases in the literature and patients cared for at our institution. We analyzed clinical, laboratory, and histologic features (including vessel size and type of inflammatory infiltrates); course of illness; biopsy procedure; and treatment. Based on the presence of localized or systemic disease at the time of disease presentation and during the follow-up, we divided patients into 3 groups: IVB (Group 1), VB with proven or indirect evidence of systemic vasculitis (Group 2), and VB with possible systemic involvement (Group 3). We identified a total of 34 cases of VB (30 from PubMed [National Library of Medicine, Bethesda, MD] and 4 from our pathology database). All patients presented with breast lesions, which were the only expression of disease in 16 (47%). Eighteen, 6, and 10 patients belonged to Group 1, 2, and 3, respectively. Constitutional symptoms were present less often in Group 1. Musculoskeletal symptoms occurred only in Groups 2 and 3. Patients in Groups 2 and 3 had higher erythrocyte sedimentation rates and lower hemoglobin levels, and also received corticosteroids more frequently than those in Group 1. No differences were found in the other analyzed parameters between groups. In summary, VB is uncommon, and in about half of the cases, occurs in the form of IVB. Histologic characteristics do not correlate with disease extent. In IVB patients, constitutional and musculoskeletal manifestations are usually absent. Such patients generally do not require systemic therapy and may be cured by resection alone.
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Affiliation(s)
- José Hernández-Rodríguez
- From the Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases (JHR, ESM, AK, GSH) and Department of Anatomic Pathology (CDT, ERR), Cleveland Clinic, Cleveland, OH
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Vrancken AFJE, Hughes RAC, Said G, Wokke JHJ, Notermans NC. Immunosuppressive treatment for non-systemic vasculitic neuropathy. Cochrane Database Syst Rev 2007; 2007:CD006050. [PMID: 17253577 PMCID: PMC11831415 DOI: 10.1002/14651858.cd006050.pub2] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
BACKGROUND Non-systemic vasculitic neuropathy is a rare disabling disease that usually has a subacute onset of progressive or relapsing-remitting sensory or sensorimotor deficits. Asymmetry, pain and weakness are key features. The diagnosis can only be made by exclusion of other causes, the absence of systemic vasculitis or other rheumatic diseases, and the demonstration of vasculitis in a nerve or a combined nerve and muscle biopsy. There is a need for efficacious therapy to prevent disease progression and to improve prognosis. OBJECTIVES To assess if immunosuppressive treatment in non-systemic vasculitic neuropathy reduces disability, and ameliorates neurological symptoms, and if such therapy can be given safely. SEARCH STRATEGY The Cochrane Neuromuscular Disease Group Trials Register (March 2006), The Cochrane Library (Issue 1, 2006), MEDLINE, EMBASE, LILACS, and ISI were searched from January 1980 until April 2006. In addition, the reference lists of relevant articles, reviews and textbooks were handsearched. SELECTION CRITERIA All randomised or quasi-randomised trials that examined the efficacy of immunosuppressive treatment for non-systemic vasculitic neuropathy at least one year after the onset of therapy were sought. Participants had to fulfill the following criteria: absence of systemic or neurological disease, exclusion of any recognised cause of the neuropathy by appropriate clinical or laboratory investigations, electrophysiological studies in agreement with axonal neuropathy, confirmation of vasculitis in a nerve or a combined nerve and muscle biopsy. The primary outcome measure was to be improvement in disability. Secondary outcome measures were to be change in the mean disability score, change in muscle strength measured with the Medical Research Council sum score, change in pain or other positive sensory symptoms, number of relapses, and adverse events. DATA COLLECTION AND ANALYSIS Two authors independently reviewed and extracted details of all potentially relevant trials. For included studies pooled relative risks and pooled weighted standardised mean differences were to be calculated to assess treatment efficacy. MAIN RESULTS Fifty-nine studies were identified and assessed for possible inclusion in the review, but all were excluded because of insufficient quality or lack of relevance. AUTHORS' CONCLUSIONS No adequate randomised or quasi-randomised controlled clinical trials have been performed on which to base treatment for non-systemic vasculitic neuropathy. Randomised trials of corticosteroids and other immunosuppressive agents are needed.
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Affiliation(s)
- A F J E Vrancken
- University Medical Center Utrecht, Department of Neurology, The Rudolf Magnus Institute for Neuroscience, Heidelberglaan 100, PO Box 85500, Utrecht, Netherlands, 3508 GA.
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Mortensen K, Lichtenberg J, Thomsen PD, Larsson LI. Spontaneous fusion between cancer cells and endothelial cells. Cell Mol Life Sci 2004; 61:2125-31. [PMID: 15316661 PMCID: PMC11138582 DOI: 10.1007/s00018-004-4200-2] [Citation(s) in RCA: 62] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Endothelial cells line the inside of blood and lymphatic vessels, and cancer cells must cross this barrier, first to gain access to the circulation, and, second, to exit and metastasize. How this occurs is incompletely understood. We now demonstrate that human cancer cells are able to fuse with endothelial cells to form hybrid cells displaying proteins and chromosomal markers characteristic of both parent cells. The hybrid cells are viable and capable of undergoing mitosis. Fusions between cancer cells and endothelial cells were shown to occur both in vitro, in co-cultures of human breast cancer cells and endothelial cells, and in vivo, following intravascular dissemination of human breast cancer cells in nude mice. These observations demonstrate a new type of cancer-endothelial cell interaction that may be of fundamental importance to the process of metastasis.
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Affiliation(s)
- K. Mortensen
- Department of Anatomy and Physiology, KVL, Gronnegaardsvej 7, 1870 Frederiksberg C Copenhagen, Denmark
| | | | - P. D. Thomsen
- Department of Anatomy and Physiology, KVL, Gronnegaardsvej 7, 1870 Frederiksberg C Copenhagen, Denmark
| | - L.-I. Larsson
- Department of Anatomy and Physiology, KVL, Gronnegaardsvej 7, 1870 Frederiksberg C Copenhagen, Denmark
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