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Wu L, Sun X, Yang Y, Liu Z, Cui L, Song X, Zeng R, Zhang H, Li F, Zhou J, Zheng W, Chen Y, Zheng Y. Optimal timing of surgical treatment for Behcet's disease aortic or peripheral artery pseudoaneurysms. J Vasc Surg 2025:S0741-5214(25)00329-5. [PMID: 39971144 DOI: 10.1016/j.jvs.2025.02.006] [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: 12/13/2024] [Revised: 01/29/2025] [Accepted: 02/05/2025] [Indexed: 02/21/2025]
Abstract
OBJECTIVE The aim of this study was to assess the optimal thresholds of surgical intervention for Behcet's disease (BD) aortic or peripheral artery pseudoaneurysms. METHODS This was a retrospective single-center study of 2138 patients with BD, including 96 patients with vascular BD (4.5%) with 151 pseudoaneurysms between January 2002 and December 2021. Factors associated with mortality were assessed using Cox regression model. The growth rate of each pseudoaneurysm was calculated based on available imaging data in a linear mixed effect model. RESULTS Patients in the surgical group (2/56) had significantly lower aneurysm-related mortality than those in the medical treatment group (10/33) (χ2 = 10.34; P = .0013). Surgical intervention (P = .009) and diameter of BD pseudoaneurysm (P = .006) were independently associated with BD aneurysm-related mortality. Rapid growth of BD pseudoaneurysm was achieved once diameter exceeded 4.0 cm for aortic pseudoaneurysm, or 2.5 cm for peripheral artery pseudoaneurysm, accompanied with high risk of rupture (Overall rupture rate: BD aortic pseudoaneurysm, ≥4.0 cm vs <4.0 cm: 63.6% vs 15.4%; BD peripheral artery pseudoaneurysm, ≥2.5 cm vs <2.5 cm: 50.9% vs 0). CONCLUSIONS This study verified the critical role of surgical intervention in reducing the mortality rate of patients with BD pseudoaneurysms. BD aortic pseudoaneurysms larger than 4.0 cm in diameter and peripheral artery pseudoaneurysms larger than 2.5 cm in diameter require prompt surgical intervention due to the remarkable increase in the growth rate and greater risk of rupture and death.
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Affiliation(s)
- Lianglin Wu
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiaoning Sun
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yisen Yang
- Department of Epidemiology and Biostatistics, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine, Peking Union Medical College, Beijing, China
| | - Zhili Liu
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Liqiang Cui
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xitao Song
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Rong Zeng
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hui Zhang
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Fangda Li
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Jingya Zhou
- Department of Medical Record, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Wenjie Zheng
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Beijing, China
| | - Yuexin Chen
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yuehong Zheng
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Department of Vascular Surgery, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
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Vlasenko AI, Topuzova MP, Malko VA, Bisaga GN, Pavlova TA, Abramova AP, Chistyakova AO, Alekseeva TM. [Challenges of differential diagnosis in the verification of Behçet's disease with neurological manifestations]. Zh Nevrol Psikhiatr Im S S Korsakova 2025; 125:108-113. [PMID: 40350737 DOI: 10.17116/jnevro2025125041108] [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] [Indexed: 05/14/2025]
Abstract
Behçet's disease (BD) is an autoimmune vasculitis of unclear etiology characterized by a wide range of systemic manifestations. The nervous system is involved in approximately 10% of patients with BD (neuro-Behçet). Diagnosis of neuro-Behçet is challenging, especially in the case of BD onset with neurological manifestations, and is based on a combination of clinical, laboratory, and neuroimaging data. To verify the diagnosis of neuro-Behçet, a wide range of conditions with similar manifestations must be ruled out: multiple sclerosis, neuromyelitis optica spectrum disorder, neurosarcoidosis, and many rare autoimmune diseases (e.g., Cogan syndrome, Susac syndrome, Vogt-Koyanagi-Harada syndrome). This article presents a brief literature review highlighting the challenges of neuro-Behçet differential diagnosis and a clinical case of a 34-year-old male patient with a criteria-based diagnosis of BD with neurological manifestations.
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Affiliation(s)
- A I Vlasenko
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - M P Topuzova
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - V A Malko
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - G N Bisaga
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - T A Pavlova
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - A P Abramova
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - A O Chistyakova
- Almazov National Medical Research Center, St. Petersburg, Russia
| | - T M Alekseeva
- Almazov National Medical Research Center, St. Petersburg, Russia
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Borelli A, Behr J, Ruggeri M, Han M, Zhou Y, Foster CS. Indications for Magnetic Resonance Imaging in Patients With Behcet Uveitis. J Neuroophthalmol 2024; 44:473-477. [PMID: 37847219 DOI: 10.1097/wno.0000000000002018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2023]
Abstract
BACKGROUND Behcet disease is a systemic vasculitis, which may involve the eyes and central nervous system. The true prevalence of neurological involvement is not precisely known but may be associated with ocular involvement. This study investigates the association between Behcet uveitis and neuro-Behcet disease. METHODS A retrospective single-center analysis was conducted for consecutive patients with Behcet uveitis at the Massachusetts Eye Research and Surgery Institution. Uveitis characteristics, neurological symptoms, fluorescein fundus angiography, and MRI results were recorded. RESULTS Our population included 108 patients with Behcet uveitis, and 26 (24.1%) were found to have neurological involvement associated with Behcet disease. Optic nerve leakage on fundus angiography and neurological symptoms were associated with an increased risk of neurological involvement. Three cases (11.5%) were nonparenchymal, while 23 (88.5%) were parenchymal with lesions in the cortex, subcortical white matter, thalamus, basal ganglia, and brainstem. CONCLUSIONS There is a high comorbidity between ocular and neurological involvement in Behcet disease. Careful assessment of neurological symptoms and baseline fluorescein fundus angiography are recommended for patients with Behcet disease. MRI has a high diagnostic yield and should be pursued if there is concern for progressive or pre-existing neurological involvement.
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Affiliation(s)
- Anna Borelli
- Massachusetts Eye Research and Surgery Institution (AB, JB, MR, MH, YZ, CSF), Waltham, Massachusetts; The Ocular Immunology and Uveitis Foundation (AB, JB, MR, MH, YZ, CSF), Waltham, Massachusetts; and Department of Ophthalmology (CSF), Harvard Medical School, Boston, Massachusetts
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Badihian S, Kiczek MP, Hajj-Ali RA. Central Nervous System Imaging in Rheumatic Diseases. Rheum Dis Clin North Am 2024; 50:559-579. [PMID: 39415368 DOI: 10.1016/j.rdc.2024.07.001] [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] [Indexed: 10/18/2024]
Abstract
Central nervous system (CNS) involvement is seen across a variety of rheumatic diseases. Common CNS manifestations include cerebrovascular events (such as ischemic and hemorrhagic strokes, cerebral venous sinus thrombosis), seizures, headaches, demyelinating lesions (such as optic neuritis, myelitis, parenchymal lesions), meningeal disease (leptomeningeal and pachymeningeal involvement, meningitis), and others. While most patients have other systemic symptoms of their rheumatic disease at the time of developing neurologic complications, some may present with CNS disease as the first symptom of their rheumatic disease. This review discusses rheumatic diseases with CNS manifestations and provides neuroimaging findings observed among affected individuals.
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Affiliation(s)
- Shervin Badihian
- Department of Neurology, Neurological Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Matthew P Kiczek
- Department of Radiology and Imaging, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Rula A Hajj-Ali
- Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Lerner College of Medicine, Center for Vasculitis Care & Research, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
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Kim J. Cardiovascular Manifestations in Behçet's Disease. Yonsei Med J 2024; 65:493-500. [PMID: 39193757 PMCID: PMC11359608 DOI: 10.3349/ymj.2023.0578] [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] [Received: 01/16/2024] [Revised: 06/03/2024] [Accepted: 06/10/2024] [Indexed: 08/29/2024] Open
Abstract
Cardiovascular involvement in Behçet's disease (BD) is considerably related to morbidity and mortality. However, the cardiovascular manifestation is sometimes difficult to distinguish from those of other causes. The suspicion of BD and proper treatment is pivotal in the management of BD. Histology demonstrates perivasculitis. Neutrophil seems to play an important role in the inflammation of BD. It is thought that inflammation causes venous thrombosis and arterial aneurysm. Characteristically, BD involves both arteries and veins of variable size in any region. Venous thrombosis needs immunosuppression, and inferior vena cava thrombosis and Budd-Chiari syndrome require intensive immunosuppressive therapy. Arterial involvement causes aneurysm which usually is treated by surgical or endovascular intervention with immunosuppression. Pulmonary artery aneurysm and cardiac involvement require multimodal managements.
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Affiliation(s)
- Jinhyun Kim
- Division of Rheumatology, Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.
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Almutamaiz AH, Alshoabi SA, Al Akhali ES, Hamid AM, Gareeballah A, Omer AM. Behçet's Syndrome With Multiple Bilateral Pulmonary Aneurysms Associated With Endomyocardial Fibrosis Presented With Pulmonary Emboli: A Case Report. Cureus 2024; 16:e66281. [PMID: 39238732 PMCID: PMC11376314 DOI: 10.7759/cureus.66281] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/06/2024] [Indexed: 09/07/2024] Open
Abstract
Behçet's syndrome (BS) is a rare chronic multisystemic inflammatory disorder of unknown etiopathogenesis. BS is classified as a vasculitis of variable vessel size, which can manifest in both arterial and venous blood vessels. BS commonly presents with mucocutaneous and ocular manifestations. Superficial and deep vein thrombosis is present in 50% of patients, with atypical venous thrombosis affecting the inferior vena cava, superior vena cava, hepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses, and right atrium and ventricle. Arterial manifestations include in situ thrombosis, pulmonary artery aneurysms, aneurysms of the abdominal aorta, and aneurysms of visceral and peripheral arteries. This article reports a new case of BS in a 28-year-old female patient who presented with severe dyspnea and hemoptysis. Echocardiography and cardiovascular magnetic resonance imaging led to the diagnosis of endomyocardial fibrosis and a large right ventricular thrombus with pulmonary embolism. Computed tomography angiography revealed multiple pulmonary aneurysms and emboli. Rare findings such as endomyocardial fibrosis and Budd-Chiari syndrome were noted. This case highlights the role of medical imaging modalities in diagnosing rare syndromes such as BS, as demonstrated in the current case.
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Affiliation(s)
- Ali H Almutamaiz
- Department of Radiology, Al-Thawra Modern General Hospital, Sanaa, YEM
- Department of Radiology, Faculty of Medicine, Sana'a University, Sanaa, YEM
| | - Sultan A Alshoabi
- Department of Diagnostic Radiology, College of Applied Medical Sciences, Taibah University, Al-Madinah Al-Munawwarah, SAU
| | - Eman S Al Akhali
- Department of Radiology, Advanced Al Razi Diagnostic Center, Sanaa, YEM
| | | | - Awadia Gareeballah
- Department of Diagnostic Radiology, College of Applied Medical Sciences, Taibah University, Al-Madinah Al-Munawwarah, SAU
| | - Awatif M Omer
- Department of Diagnostic Radiology, College of Applied Medical Sciences, Taibah University, Al-Madinah Al-Munawwarah, SAU
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Yoshimoto K, Kobayashi T, Matsuoka H, Nishimura N, Kawashima H, Yoneima R, Tsushima E, Ono S, Nishio K. Neuro-Behçet's disease with atypical subcortical nodular lesions: A case report and treatment approach. Mod Rheumatol Case Rep 2024; 8:348-351. [PMID: 38092418 DOI: 10.1093/mrcr/rxad070] [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: 10/10/2023] [Revised: 11/22/2023] [Accepted: 11/27/2023] [Indexed: 07/09/2024]
Abstract
Neuro-Behçet's disease (NB) is a rare complication of Behçet's disease (BD) characterised by central nervous system involvement. While NB typically presents with brainstem lesions, we report an unusual case of NB in a 27-year-old male with multiple subcortical nodular brain lesions but without brainstem, thalamic, or basal ganglia involvement, making this presentation exceptionally rare. The patient had a prior diagnosis of BD and was HLA-B51 positive. He presented with a sudden loss of consciousness, which was attributed to a seizure. Imaging studies showed low-density areas in the white matter of the bilateral temporal lobes and the right frontoparietal lobe on brain CT. Cerebrospinal fluid examination indicated elevated initial pressure and protein concentration, along with increased interleukin-6. Despite presenting with nodular brain lesions, distinguishing between NB and infectious diseases such as tuberculosis (TB) was challenging, and required brain biopsy revealing vasculitis. However, even with this biopsy result, TB could not be ruled out, so TB was treated at the same time. Treatment with anti-TB drugs and standard steroid therapy initially failed to improve the patient's condition. However, increasing the steroid dosage considering the increased steroid degradation by rifampicin, including pulse therapy with 2 g of methylprednisolone, followed by 18 mg of betamethasone, led to remission of the nodular brain lesions and resolution of the nasopharyngeal ulcer. This case highlights the diagnostic challenge of differentiating between NB and TB based on imaging alone and the potential efficacy of high-dose steroid therapy in cases of steroid-resistant NB with subcortical nodular brain lesions.
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Affiliation(s)
- Kiyomi Yoshimoto
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Tadanao Kobayashi
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
- Department of General Medicine, Uda Municipal Hospital, Uda, Nara, Japan
| | - Hidetoshi Matsuoka
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Nobushiro Nishimura
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Hiromasa Kawashima
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Ryo Yoneima
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Emiko Tsushima
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
- Yawaragi clinic, Sango, Nara, Japan
| | - Shiro Ono
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
| | - Kenji Nishio
- Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara, Japan
- Department of General Medicine, Uda Municipal Hospital, Uda, Nara, Japan
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Ramegowda R, Singhal M, Gulati A, Samanta J, Singh H, Sharma V, Sharma A, Gupta P. Autoimmune disorders of the gastrointestinal tract: Review of radiological appearances. Curr Probl Diagn Radiol 2024; 53:259-270. [PMID: 37923635 DOI: 10.1067/j.cpradiol.2023.10.002] [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: 06/08/2023] [Revised: 09/26/2023] [Accepted: 10/18/2023] [Indexed: 11/07/2023]
Abstract
Autoimmune gastrointestinal (GI) disorders comprise a heterogeneous group of diseases with non-specific clinical manifestations. These are divided into primary and secondary. A high index of clinical suspicion complemented with endoscopic and radiological imaging may allow early diagnosis. Due to the relatively low incidence of autoimmune disorder, the imaging literature is sparse. In this review, we outline the pathogenesis, classification, and imaging appearances of autoimmune GI disorders.
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Affiliation(s)
- Rajath Ramegowda
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Manphool Singhal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Ajay Gulati
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Jayanta Samanta
- Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Harjeet Singh
- Department of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Vishal Sharma
- Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Aman Sharma
- Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Pankaj Gupta
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
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Lin L, Zhang D. Behçet Disease Periaortic Pseudoaneurysms Connect with Pulmonary Artery. Radiology 2024; 310:e232540. [PMID: 38411512 DOI: 10.1148/radiol.232540] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/28/2024]
Affiliation(s)
- Lu Lin
- From the Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Shuai Fu Yuan No. 1, Dongcheng District, Beijing 100730, China
| | - Daming Zhang
- From the Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Shuai Fu Yuan No. 1, Dongcheng District, Beijing 100730, China
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Manuelyan Z, Butt E, Parupudi S. Gastrointestinal Behçet's disease: Manifestations, diagnosis, and management. Dis Mon 2024; 70:101674. [PMID: 38185603 DOI: 10.1016/j.disamonth.2023.101674] [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] [Indexed: 01/09/2024]
Abstract
Behçet's disease (BD) is a rare, inflammatory vascular disorder with recurrent oral and genital aphthous ulcers, along with ocular and cutaneous manifestations. Gastrointestinal (GI) BD may involve any portion of the GI tract. However, it is commonly described in the terminal ileum, followed by the ileocecal region. Diagnosis is challenging given lack of pathognomonic tests; therefore, it is based on clinical criteria. Management of intestinal BD includes different classes of medications including corticosteroids, 5-aminosalicylic acid, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody agents. In this review, we aim to focus on intestinal BD and provide details of clinical manifestations, diagnosis and therapeutic options of intestinal BD from gastroenterology viewpoint.
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Affiliation(s)
- Zara Manuelyan
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, The University of Texas Medical Branch at Galveston, Galveston, TX 77550, USA
| | - Edward Butt
- Department of Internal Medicine, The University of Texas Medical Branch at Galveston, 301 University Blvd, Galveston, TX 77550, USA.
| | - Sreeram Parupudi
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, The University of Texas Medical Branch at Galveston, Galveston, TX 77550, USA
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Dafer RM, Osteraas ND. Non-atherosclerotic Cerebral Vasculopathies. ISCHEMIC STROKE THERAPEUTICS 2024:271-299. [DOI: 10.1007/978-3-031-49963-0_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Ayoade F, Hernandez S, Montreuil N, Drews-Elger K, Quiroz T, Sternberg CA. Case report: A case of neuro-Behçet's syndrome presenting as brain stem mass lesions. Front Neurol 2023; 14:1218680. [PMID: 37583955 PMCID: PMC10424439 DOI: 10.3389/fneur.2023.1218680] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2023] [Accepted: 07/07/2023] [Indexed: 08/17/2023] Open
Abstract
Neuro-Behçet's syndrome, a severe and rare manifestation of Behçet's disease (BD), can be misdiagnosed due to its challenging clinical presentation. This article presents the case of a 20-year-old cis-gender male with intermittent fever, bilateral uveitis, and neurological symptoms who was found to have multiple brain stem mass lesions on brain imaging. A careful medical history elicited recurrent painful oral and genital ulcerations which were important in making the correct diagnosis. As there are no validated criteria or definite set of tests available to confirm neuro-Behçet's disease, the diagnosis is often established by exclusion after ruling out other potential etiologies. In our case, after an extensive negative workup for infectious, neuro-degenerative and malignant etiologies combined with the patient's medical history, a diagnosis of Behçet's disease with neurological involvement (neuro-Behçet's syndrome) was made. High doses of steroids were given, and the patient had a favorable outcome. Repeated magnetic resonance imaging of the brain 2 years later showed no new brain lesions. Neuro-Behçet's disease should be included as a differential diagnosis of unexplained brain stem lesions in the right clinical context. In these situations, providers should obtain medical histories related to genital and oral ulcers and eye problems as these may help to narrow down the diagnosis. The clinical presentation and challenges of this uncommon presentation of BD including a brief literature review of neuro-Behçet's disease with brain stem mass lesions are discussed in this case study.
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Affiliation(s)
- Folusakin Ayoade
- University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States
| | - Salma Hernandez
- University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States
| | - Nadine Montreuil
- University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States
| | - Katherine Drews-Elger
- University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States
- Department of Pathology and Laboratory Medicine, Jackson Health System, Miami, FL, United States
| | - Tanya Quiroz
- Division of Infectious Disease, Department of Medicine, Jackson Health System, Miami, FL, United States
| | - Candice A. Sternberg
- University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States
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13
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Marchiori E, Hochhegger B, Zanetti G. Multiple vascular nodules. J Bras Pneumol 2023; 49:e20230173. [PMID: 37493792 PMCID: PMC10578903 DOI: 10.36416/1806-3756/e20230173] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/27/2023] Open
Affiliation(s)
- Edson Marchiori
- . Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ) Brasil
| | - Bruno Hochhegger
- . Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre (RS) Brasil
| | - Gláucia Zanetti
- . Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ) Brasil
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14
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Polat G, Tezcan MA, Polat M, Barutçugil F, Bayram T, Sade R, Kudaş Ö, Pirimoğlu RB, İsmailova E, Yalçın A, Meletlioğlu E, Kahraman M. Quantitative analysis of lung parenchymal involvement in Behçet's disease by the novel automatic program. Int J Rheum Dis 2023; 26:917-921. [PMID: 36946548 DOI: 10.1111/1756-185x.14673] [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: 01/13/2023] [Revised: 02/18/2023] [Accepted: 03/10/2023] [Indexed: 03/23/2023]
Abstract
OBJECTIVES We aimed to quantitatively analyze lung parenchymal changes in Behçet's patients and to detect early parenchymal quantitative changes that occur in the absence of positive visual radiological findings. METHODS In our study, a total of 31 patients with Behçet's disease, 17 with positive radiological findings and 14 patients without positive radiological findings, and a control group of 33 individuals were evaluated. The automatic program determined lung volumes, lung densities, and opacity volume percentages by evaluating the contrast-enhanced lung computed tomography scans. RESULTS The total lung volume was 3632.98 ± 1100.53 mL in the Behçet's disease patient group and 4925.70 ± 1098.88 mL in the control group, and there was a significant decrease in the total lung volume in Behçet's disease patients (P < 0.0001). The mean lung density was -723.73 ± 65.16 in the Behçet's disease patient group and -767.35 ± 41.17 in the control group, and there was a significant increase in the mean density in the Behçet's patients (P = 0.0023). A significantly higher correlation was observed between lung density and lung volume in Behçet's patients (P < 0.0001, r = -0.795). The lung volume of Behçet's disease patients with negative radiological findings was significantly lower than that of the control group (P = 0.0342). CONCLUSIONS Lung volumetric changes in Behçet's disease patients are the quantitative data most affected by the disease. In addition, lung volume decreases in Behçet's patients who do not have visual parenchymal involvement.
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Affiliation(s)
- Gökhan Polat
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | | | - Merve Polat
- Department of Health Physics, Health Sciences Institute, Karadeniz Technical University, Trabzon, Turkey
| | - Furkan Barutçugil
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - Tuncay Bayram
- Department of Health Physics, Health Sciences Institute, Karadeniz Technical University, Trabzon, Turkey
- Department of Physics, Karadeniz Technical University, Trabzon, Turkey
| | - Recep Sade
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - Özlem Kudaş
- Department of Rheumatology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - R Berhan Pirimoğlu
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - Elshad İsmailova
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - Ahmet Yalçın
- Department of Radiology, Medical Faculty, Ataturk University, Erzurum, Turkey
| | - Emrah Meletlioğlu
- Department of Mechanical Engineering, Institute of Science, Ataturk University, Erzurum, Turkey
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15
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Giannessi C, Smorchkova O, Cozzi D, Zantonelli G, Bertelli E, Moroni C, Cavigli E, Miele V. Behçet's Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement. Diagnostics (Basel) 2022; 12:2868. [PMID: 36428928 PMCID: PMC9689730 DOI: 10.3390/diagnostics12112868] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2022] [Revised: 11/13/2022] [Accepted: 11/15/2022] [Indexed: 11/22/2022] Open
Abstract
Behcet's disease (BD) is a chronic systemic inflammatory disorder characterized by underlying chronic vasculitis of both large- and small-caliber vessels. Thoracic involvement in BD can occur with various types of manifestations, which can be detected with contrast-enhanced MSCT scanning. In addition, MR can be useful in diagnosis. Characteristic features are aneurysms of the pulmonary arteries that can cause severe hemoptysis and SVC thrombosis that manifests as SVC syndrome. Other manifestations are aortic and bronchial artery aneurysms, alveolar hemorrhage, pulmonary infarction, and rarely pleural effusion. Achieving the right diagnosis of these manifestations is important for setting the correct therapy and improving the patient's outcome.
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Affiliation(s)
| | | | - Diletta Cozzi
- Department of Emergency Radiology, Careggi University Hospital, 50134 Florence, Italy
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16
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Abstract
RATIONALE Behçet's disease (BD) is an autoimmune disease presented with recurrent oral aphthous ulcers, genital ulcers, uveitis and skin lesions. The cutaneous manifestations are often refractory and more resistant to conventional therapy. This report provides a new treatment of low-dose interleukin-2 (IL-2) with refractory BD patient. PATIENT CONCERNS A 37-year-old female patient experienced oral ulcer and erythema nodosum on the right leg for over 12 months and resisted to Methylprednisolone and Thalidomide. DIAGNOSES The patient suffered from recurrent painful oral ulceration and an erythema nodosum. Pathergy test is also positive. Thus, we diagnosed her as BD according to the International Criteria for Behçet's Disease (ICBD). INTERVENTIONS The patient took Methylprednisolone 8 mg qd, Thalidomide 50 mg qn and Hydroxychloroquine 200 mg bid successively as treatment. However, the medicine didn't take effect. Finally, this patient was given low-dose IL-2 intramuscular injection qod for 3 months. OUTCOMES Oral ulcers and the erythema disappeared and the patient has been symptom-free for 6 months. LESSONS low-dose IL-2 is a safe and effective treatment for refractory BD.
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Affiliation(s)
- Wenyan Zhou
- Department of Rheumatology & Immunology, Peking University People’s Hospital, Beijing, China
| | - Tian Liu
- Department of Rheumatology & Immunology, Peking University People’s Hospital, Beijing, China
- Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China
| | - Xian Xiao
- Department of Rheumatology & Immunology, Peking University People’s Hospital, Beijing, China
- Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China
| | - Jing He
- Department of Rheumatology & Immunology, Peking University People’s Hospital, Beijing, China
- Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China
- *Correspondence: Jing He, Department of Rheumatology and Immunology, Peking University People’s Hospital Beijing, ChinaBeijing 100044, China (e-mail: )
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17
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Amouei M, Momtazmanesh S, Kavosi H, Davarpanah AH, Shirkhoda A, Radmard AR. Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Insights Imaging 2022; 13:143. [PMID: 36057741 PMCID: PMC9440973 DOI: 10.1186/s13244-022-01284-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Accepted: 08/04/2022] [Indexed: 11/21/2022] Open
Abstract
Diagnosis of intestinal vasculitis is often challenging due to the non-specific clinical and imaging findings. Vasculitides with gastrointestinal (GI) manifestations are rare, but their diagnosis holds immense significance as late or missed recognition can result in high mortality rates. Given the resemblance of radiologic findings with some other entities, GI vasculitis is often overlooked on small bowel studies done using computed tomography/magnetic resonance enterography (CTE/MRE). Hereon, we reviewed radiologic findings of vasculitis with gastrointestinal involvement on CTE and MRE. The variety of findings on MRE/CTE depend upon the size of the involved vessels. Signs of intestinal ischemia, e.g., mural thickening, submucosal edema, mural hyperenhancement, and restricted diffusion on diffusion-weighted imaging, are common in intestinal vasculitis. Involvement of the abdominal aorta and the major visceral arteries is presented as concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels. Such findings can be observed particularly in large- and medium-vessel vasculitis. The presence of extra-intestinal findings, including within the liver, kidneys, or spleen in the form of focal areas of infarction or heterogeneous enhancement due to microvascular involvement, can be another radiologic clue in diagnosis of vasculitis. The link between the clinical/laboratory findings and MRE/CTE abnormalities needs to be corresponded when it comes to the diagnosis of intestinal vasculitis.
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Affiliation(s)
- Mehrnam Amouei
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
| | - Sara Momtazmanesh
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
| | - Hoda Kavosi
- Department of Rheumatology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Amir H. Davarpanah
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, USA
| | - Ali Shirkhoda
- Department of Radiological Science, University of California at Irvine, Irvine, USA
| | - Amir Reza Radmard
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
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18
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Imaging More than Skin-Deep: Radiologic and Dermatologic Presentations of Systemic Disorders. Diagnostics (Basel) 2022; 12:diagnostics12082011. [PMID: 36010360 PMCID: PMC9407377 DOI: 10.3390/diagnostics12082011] [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: 07/27/2022] [Revised: 08/15/2022] [Accepted: 08/17/2022] [Indexed: 11/22/2022] Open
Abstract
Background: Cutaneous manifestations of systemic diseases are diverse and sometimes precede more serious diseases and symptomatology. Similarly, radiologic imaging plays a key role in early diagnosis and determination of the extent of systemic involvement. Simultaneous awareness of skin and imaging manifestations can help the radiologist to narrow down differential diagnosis even if imaging findings are nonspecific. Aims: To improve diagnostic accuracy and patient care, it is important that clinicians and radiologists be familiar with both cutaneous and radiologic features of various systemic disorders. This article reviews cutaneous manifestations and imaging findings of commonly encountered systemic diseases. Conclusions: Familiarity with the most disease-specific skin lesions help the radiologist pinpoint a specific diagnosis and consequently, in preventing unnecessary invasive workups and contributing to improved patient care.
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19
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Law N, Quencer K, Kaufman C, Iravani A, Hardman R, Smith T. Embolization of pulmonary artery aneurysms in a patient with Behçet’s disease complicated by coil erosion into the airway. J Vasc Surg Cases Innov Tech 2022; 8:193-195. [PMID: 35402753 PMCID: PMC8989708 DOI: 10.1016/j.jvscit.2022.02.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2021] [Accepted: 02/04/2022] [Indexed: 11/15/2022] Open
Affiliation(s)
- Nicole Law
- Department of Interventional Radiology, University of Utah, Salt Lake City, UT
| | - Keith Quencer
- Department of Interventional Radiology, University of Utah, Salt Lake City, UT
| | - Claire Kaufman
- Department of Interventional Radiology, University of Utah, Salt Lake City, UT
| | - Aidin Iravani
- Department of Pulmonology, University of Utah, Salt Lake City, UT
| | | | - Tyler Smith
- Department of Interventional Radiology, University of Utah, Salt Lake City, UT
- Correspondence: Tyler Smith, MD, Department of Interventional Radiology, University of Utah, 30 North 1900 East #1A071, Salt Lake City, UT 84132-2140
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20
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Choi E, Mathews LM, Paik J, Corretti MC, Wu KC, Michos ED, Hays AG, Mukherjee M. Multimodality Evaluation of Aortic Insufficiency and Aortitis in Rheumatologic Diseases. Front Cardiovasc Med 2022; 9:874242. [PMID: 35497991 PMCID: PMC9039512 DOI: 10.3389/fcvm.2022.874242] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2022] [Accepted: 03/21/2022] [Indexed: 12/17/2022] Open
Abstract
Aortic insufficiency is commonly observed in rheumatologic diseases such as ankylosing spondylitis, systemic lupus erythematosus, antiphospholipid syndrome, Behçet's disease, granulomatosis with polyangiitis, and Takayasu arteritis. Aortic insufficiency with an underlying rheumatologic disease may be caused by a primary valve pathology (leaflet destruction, prolapse or restriction), annular dilatation due to associated aortitis or a combination of both. Early recognition of characteristic valve and aorta morphology on cardiac imaging has both diagnostic and prognostic importance. Currently, echocardiography remains the primary diagnostic tool for aortic insufficiency. Complementary use of computed tomography, cardiac magnetic resonance imaging and positron emission tomography in these systemic conditions may augment the assessment of underlying mechanism, disease severity and identification of relevant non-valvular/extracardiac pathology. We aim to review common rheumatologic diseases associated with aortic insufficiency and describe their imaging findings that have been reported in the literature.
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Affiliation(s)
- Eunjung Choi
- Dartmouth-Hitchcock Medical Center, Heart and Vascular Center, Lebanon, NH, United States
| | - Lena M. Mathews
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
| | - Julie Paik
- Division of Rheumatology, Johns Hopkins University, Baltimore, MD, United States
| | - Mary C. Corretti
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
| | - Katherine C. Wu
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
| | - Erin D. Michos
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
| | - Allison G. Hays
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
| | - Monica Mukherjee
- Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States
- *Correspondence: Monica Mukherjee
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21
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Saha BK, Chong WH, Milman NT. Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach. Clin Rheumatol 2022; 41:325-336. [PMID: 34491458 DOI: 10.1007/s10067-021-05895-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2020] [Revised: 08/20/2021] [Accepted: 08/23/2021] [Indexed: 12/11/2022]
Abstract
This narrative review provides an overview of diffuse alveolar hemorrhage (DAH) associated with rheumatologic and autoimmune diseases and their differentiation from idiopathic pulmonary hemosiderosis (IPH). Relevant immunologic diseases associated with DAH are discussed, and a diagnostic flowchart is proposed to establish a "definitive" diagnosis of IPH within the spectrum of DAH. IPH is a rare cause of recurrent DAH both in children and adults. In adults, a definitive diagnosis of IPH requires a lung biopsy and histopathologic examination demonstrating intraalveolar hemorrhage, hemosiderin-laden macrophages, and a variable degree of fibrosis in the absence of both capillaritis and cellular inflammation. The presence of small vessel vasculitis points towards immunologic, well-differentiated, or sometimes undifferentiated rheumatologic diseases. However, it is essential to recognize that many rheumatologic diseases may in the initial phase present with DAH without any evidence of capillaritis, thus mimicking IPH. Although not definitely established, it is likely that immunologic processes are involved in IPH, and we, therefore, suggest the consideration of a more suitable term for the disease, e.g., "Immune-mediated Pulmonary Hemosiderosis" to acknowledge the aberrancy in the immune parameters and a positive response to immunosuppressive therapy.
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Affiliation(s)
- Biplab K Saha
- Division of Pulmonary and Critical Care Medicine, Ozarks Medical Center, West Plains, MO, USA.
| | - Woon H Chong
- Division of Pulmonary and Critical Care Medicine, Albany Medical Center, Albany, NY, USA
| | - Nils T Milman
- Department of Clinical Biochemistry, Næstved Hospital, University College Zealand, 4700, Næstved, Denmark
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22
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Neuro-Behçet - Clinical and radiological findings in a Patient of Sub-saharan African origin. Radiol Case Rep 2022; 17:592-598. [PMID: 34976265 PMCID: PMC8688177 DOI: 10.1016/j.radcr.2021.11.046] [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: 11/11/2021] [Accepted: 11/21/2021] [Indexed: 11/21/2022] Open
Abstract
Behçet's disease is a rare, systemic variable vessel vasculitis mostly seen in patients from the Middle East, Northern Africa and Central Asia. Neuro-Behçet disease (NBD) is often diagnosed in patients with known Behçet's disease who present with neurological symptoms and radiological features of central nervous system involvement. There are very few cases with neuro-Behçet reported from Sub-Saharan Africa in the literature. We report a case of severe parenchymal neuro-Behçet with pseudo-tumoral brainstem lesions in a young male patient of South African origin.
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23
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Qaseem Y, Cassidy F, Aganovic L, Purysko A, Mirza S, Vahdat N. Renovascular involvement of systemic vascular disease: a pictorial review. Abdom Radiol (NY) 2022; 47:3531-3545. [PMID: 35796773 PMCID: PMC9261205 DOI: 10.1007/s00261-022-03591-5] [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: 02/10/2022] [Revised: 06/10/2022] [Accepted: 06/14/2022] [Indexed: 01/18/2023]
Abstract
Like many solid organs, the kidneys are susceptible to a wide variety of systemic vascular diseases. Comprising a significant subset of these diseases are the vasculitides, broadly encompassing numerous inflammatory conditions of the blood vessels. However, many of these conditions are non-vasculitic and non-inflammatory, and differentiation of these entities is crucial to guide the initiation of proper therapy. These non-vasculitic diseases include coagulopathic conditions leading to vascular complications, hemolysis, and hematogenous processes that can affect multiple organ systems. These systemic diseases can result in both macrovascular and microvascular pathology, involving the arteries, veins, and smaller vessels, and management of these conditions can differ significantly depending upon the underlying pathophysiology. Because the clinical manifestations of these disease processes can be heterogeneous, ranging from renal dysfunction to life-threatening hemorrhage, proper recognition of these entities is essential to help guide clinicians to the correct diagnosis and prevent potentially disastrous complications. Many of these systemic vascular processes can be detected by non-invasive imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), and identification of their characteristic renal manifestations by radiologists is a critical component of patient care. This review covers a variety of these diseases and their imaging manifestations, to aid in their recognition and better equip radiologists to provide vital diagnostic information that can optimize patient care.
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Affiliation(s)
- Yousuf Qaseem
- grid.266100.30000 0001 2107 4242Department of Radiology, University of California San Diego Health, San Diego, CA USA
| | - Fiona Cassidy
- grid.266100.30000 0001 2107 4242Department of Radiology, University of California San Diego Health, San Diego, CA USA ,Department of Radiology, Veteran Administration Healthcare System, San Diego, CA USA
| | - Lejla Aganovic
- grid.266100.30000 0001 2107 4242Department of Radiology, University of California San Diego Health, San Diego, CA USA ,Department of Radiology, Veteran Administration Healthcare System, San Diego, CA USA
| | - Andrei Purysko
- grid.239578.20000 0001 0675 4725Department of Radiology, Cleveland Clinic Foundation, Cleveland, OH USA
| | - Sara Mirza
- grid.266100.30000 0001 2107 4242Department of Radiology, University of California San Diego Health, San Diego, CA USA
| | - Noushin Vahdat
- grid.266100.30000 0001 2107 4242Department of Radiology, University of California San Diego Health, San Diego, CA USA ,Department of Radiology, Veteran Administration Healthcare System, San Diego, CA USA
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24
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Yazgan Ç, Ertürk H, Taşkın A. Imaging Features of Thoracic Manifestations of Behçet's Disease: Beyond Pulmonary Artery Involvement. Curr Med Imaging 2021; 17:996-1002. [PMID: 33438546 PMCID: PMC8653419 DOI: 10.2174/1573405617999210112193856] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2020] [Revised: 11/05/2020] [Accepted: 11/12/2020] [Indexed: 11/22/2022]
Abstract
Background Behçet’s disease is a chronic multisystemic vasculitis affecting vessels of different sizes in various organs. Thoracic manifestations of the disease show a wide spectrum involving a variety of anatomic structures within the chest. However, pulmonary artery involvement is a typical manifestation of the disease that contributes significantly to mortality in patients. The study aimed to analyze CT features of thoracic manifestations, particularly pulmonary artery involvement, and to quantitatively assess bronchial arteries in Behçet’s disease. Methods Patients with Behçet’s disease who underwent CT scans for suspected thoracic involvement between 2010 and 2018 were included. CT findings of 52 patients were retrospectively analyzed for thoracic manifestations of the disease. Bronchial arteries were assessed regarding diameter in patients with/without pulmonary artery involvement. The pulmonary symptoms were noted. Results Of the 52 patients, 67% had thoracic manifestations including pulmonary artery involvement, parenchymal changes, superior vena cava thrombosis, and intracardiac thrombus. Pulmonary artery involvement was observed in 50% of the cohort. Peripheral pulmonary arteries (77%) were the most commonly affected branches, followed by lobar (42%) and central (35%) pulmonary arteries. Other thoracic findings were significantly correlated with pulmonary artery involvement (p<0.05). Compared to patients without pulmonary artery involvement, those with pulmonary artery involvement had a higher bronchial artery diameter (p<0.05) and occurrence rate of dilated bronchial arteries. Conclusion Involvement of peripheral pulmonary arteries is frequently encountered in Behçet’s disease and it can resemble pulmonary nodules. Dilated bronchial arteries, which can be observed in cases of pulmonary artery involvement, should be considered in patients with hemoptysis.
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Affiliation(s)
- Çisel Yazgan
- Faculty of Medicine, Department of Radiology, Kastamonu University, Kastamonu, Turkey
| | - Hakan Ertürk
- Department of Radiology, Ataturk Chest Diseases and Chest Surgery Education and Research Hospital, 06280 Ankara, Turkey
| | - Ayşenaz Taşkın
- Department of Chest Diseases, Ataturk Chest Diseases and Chest Surgery Education and Research Hospital, 06280 Ankara, Turkey
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25
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Multimodality Imaging of Large Vessel Vasculitis, From the AJR Special Series on Inflammation. AJR Am J Roentgenol 2021; 218:213-222. [PMID: 34232695 DOI: 10.2214/ajr.21.26150] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
Large vessel vasculitis (LVV) is a non-infectious inflammation of the large vessels, including the aorta and its main branches. Imaging plays an essential role in diagnosing LVV, given the challenges of tissue biopsy. This article reviews the types of LVV, and the multimodality imaging tools available to establish the diagnosis in patients with LVV. The cornerstone of imaging diagnosis is morphologic assessment using grey-scale ultrasound, combined grey-scale US with color Doppler US, CTA, or MRA. In the last decade, substantial progress has been made in functional and molecular imaging with FDG PET/CT for the diagnosis of LVV. Investigation is ongoing to develop novel MRA techniques and new PET tracers to assess disease activity and to differentiate the various vasculitides. An algorithm is provided to guide imaging technique selection based on the patient's specific clinical presentation.
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26
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Neuroimaging findings in rheumatologic disorders. J Neurol Sci 2021; 427:117531. [PMID: 34130065 DOI: 10.1016/j.jns.2021.117531] [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: 03/09/2021] [Revised: 05/12/2021] [Accepted: 06/02/2021] [Indexed: 01/06/2023]
Abstract
Patients with rheumatological diseases may present with neurological manifestations of peripheral and/or central nervous system (CNS). Symptoms may be related to underlying rheumatological disease or CNS effects of immune-modulating drugs. Early diagnosis and therapy may help prevent serious complications. Magnetic resonance imaging (MRI), given its excellent soft tissue details, is the preferred imaging modality when evaluating patients with rheumatological disease and suspected CNS involvement. We present a review of the neuroimaging manifestations of various rheumatic diseases with emphasis on the imaging findings on MRI.
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27
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Xie WX, Jiang HT, Shi GQ, Yang LN, Wang H. Behcet’s disease manifesting as esophageal variceal bleeding: A case report. World J Clin Cases 2021; 9:2854-2861. [PMID: 33969069 PMCID: PMC8058672 DOI: 10.12998/wjcc.v9.i12.2854] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2020] [Revised: 01/13/2021] [Accepted: 02/24/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Behcet’s disease (BD) is a chronic disease characterized by oral and vulvar ulcers as well as eye and skin damage and involves multiple systems. It presents as an alternating process of repeated attacks and remissions. Esophageal venous rupture and bleeding caused by BD is rarely reported at home and abroad. This paper reports a case of bleeding from oesophageal varices caused by BD, aiming to provide an additional dimension for considering the cause of bleeding from esophageal varices in the future.
CASE SUMMARY A 38-year-old female patient was admitted due to a gradual increase in shortness of breath and chest tightness after the activity, and was admitted to our hospital for treatment. After admission, relevant examinations showed that the patient had multiple blood clots. Four days after admission, she suddenly experienced massive hematemesis. Emergency esophagogastroduodenoscopy revealed bleeding from esophageal and gastric varices. The patient had no history of viral hepatitis or drinking habits, and no history of special genetic diseases or congenital vascular diseases. There is no obvious abnormality in liver function. After reviewing the medical history, it was found that the patient had recurred oral ulcers since childhood, ulcers were visible in the perineum during menstruation, and there was an intermittent red nodular rash and uveitis. The current skin acupuncture reaction is positive, combined with the evaluation of the external hospital and our hospital, the main diagnosis is BD. She received methylprednisolone, cyclophosphamide, immunomodulation, acid suppression, gastric protection, and anticoagulation and anti-infection treatments, and was discharged from the hospital. During the 1-year follow-up period, the patient did not vomit blood again.
CONCLUSION This case highlights bleeding from esophageal varices caused by BD, aiming to provide an additional dimension concerning the cause of bleeding from esophageal varices in the future.
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Affiliation(s)
- Wen-Xing Xie
- Department of Gastroenterology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
| | - Hai-Tao Jiang
- Department of Gastroenterology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
| | - Guo-Qing Shi
- Department of Gastroenterology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
| | - Li-Na Yang
- Department of Gastroenterology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
| | - Hong Wang
- Department of Gastroenterology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
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28
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Aghayev A, Steigner ML. Systemic vasculitides and the role of multitechnique imaging in the diagnosis. Clin Radiol 2021; 76:488-501. [PMID: 33812649 DOI: 10.1016/j.crad.2021.03.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2020] [Accepted: 03/02/2021] [Indexed: 11/17/2022]
Abstract
Vasculitis, a systemic disease characterised by inflammation of the blood vessels, remains challenging to diagnose and manage. Vessel size has been the basis for classifying systemic vasculitides. Imaging plays a vital role in diagnosing this challenging disease. This review article aims (a) to summarise up-to-date literature in this field, as well as include classification updates and (b) to review available imaging techniques, recent advances, and emphasis on imaging findings to diagnose large vessel vasculitides.
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Affiliation(s)
- A Aghayev
- Cardiovascular Imaging Program, Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
| | - M L Steigner
- Cardiovascular Imaging Program, Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
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29
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Chauhan A, Thomas R. Magnetic Resonance Imaging Appearance of Erythema Nodosum: A Case Report. J Radiol Case Rep 2021; 15:21-29. [PMID: 33717404 DOI: 10.3941/jrcr.v15i1.3919] [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] [Indexed: 11/15/2022] Open
Abstract
Erythema nodosum (EN) is the commonest inflammation of the subcutaneous fat tissue (panniculitis). Erythema nodosum (EN) requires an interdisciplinary approach and exclusion of all underlying causes. We present a case of an 18-year-old female with a history of recurrent streptococcal infections over the years, who developed pain and swelling in the left ankle. To evaluate the persistent ankle swelling, the physician ordered a magnetic resonance imaging (MRI) of the left lower extremity. The MRI appearance of EN has not been described in detail in the literature so far.
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Affiliation(s)
- Ankita Chauhan
- University of Tennessee Health Science Center/Le Bonheur Children's Hospital, Memphis, TN, USA
| | - Richard Thomas
- Department of Radiology, John R. Oishei Children's Hospital, Buffalo, USA
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30
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Sekkat A, Bouchal S, Charifi Y, Hajjam KE, Alaoui YL, Belahsen MF, Maaroufi M, Alami B. Pulmonary and intracranial miliary tuberculosis secondary to Behçet's anti-TNF alfa treatment. Radiol Case Rep 2020; 16:338-342. [PMID: 33312320 PMCID: PMC7721595 DOI: 10.1016/j.radcr.2020.11.029] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2020] [Revised: 11/13/2020] [Accepted: 11/15/2020] [Indexed: 12/28/2022] Open
Abstract
Behçet disease is a rare vasculitis that affects vessels of different body parts, causing different kinds of manifestations. We report a case of a 47 years old woman who had a tumor necrosis factor alfa blocker prescription due to a Behçet's disease relapse. The patient then developed a cerebral and pulmonary tuberculous miliary due to immunodeficiency. The aim of this work is to show that tuberculosis infection is a common complication of the administration of tumor necrosis factor alfa blocker, and the importance to perform a tuberculosis screening before starting the treatment.
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Affiliation(s)
- Asmae Sekkat
- Radiology department, Hassan II University Hospital, Fez, Morocco
| | - Siham Bouchal
- Neurology department, Hassan II University Hospital, Fez, Morocco
| | - Yahya Charifi
- Radiology department, Hassan II University Hospital, Fez, Morocco
| | - Khalid El Hajjam
- Radiology department, Hassan II University Hospital, Fez, Morocco
| | | | | | | | - Badr Alami
- Radiology department, Hassan II University Hospital, Fez, Morocco
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Gray BR, Agarwal A, Tann M, Koontz NA. PET and SPECT Imaging of Brain Neoplasia Mimics. Semin Ultrasound CT MR 2020; 41:541-550. [DOI: 10.1053/j.sult.2020.08.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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Aljizeeri A, Small G, Malhotra S, Buechel R, Jain D, Dwivedi G, Al-Mallah MH. The role of cardiac imaging in the management of non-ischemic cardiovascular diseases in human immunodeficiency virus infection. J Nucl Cardiol 2020; 27:801-818. [PMID: 30864047 DOI: 10.1007/s12350-019-01676-1] [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: 02/04/2019] [Accepted: 02/07/2019] [Indexed: 10/27/2022]
Abstract
Infection with human immunodeficiency virus (HIV) has become the pandemic of the new century. About 36.9 million people are living with HIV worldwide. The introduction of antiretroviral therapy in 1996 has dramatically changed the global landscape of HIV care, resulting in significantly improved survival and changing HIV to a chronic disease. With near-normal life expectancy, contemporary cardiac care faces multiple challenges of cardiovascular diseases, disorders specific to HIV/AIDS, and those related to aging and higher prevalence of traditional risk factors. Non-ischemic cardiovascular diseases are major components of cardiovascular morbidity and mortality in HIV/AIDS. Non-invasive cardiac imaging plays a pivotal role in the management of these diseases. This review summarizes the non-ischemic presentation of the HIV cardiovascular spectrum focusing on the role of cardiac imaging in the management of these disorders.
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Affiliation(s)
- Ahmed Aljizeeri
- King Abdulaziz Cardiac Center, Ministry of National Guard-Health Affaire, Riyadh, Saudi Arabia
- King Abdullah International Medical Research Center, Riyadh, Saudi Arabia
- King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
| | - Gary Small
- Division of Cardiology, University of Ottawa Heart Institute, Ottawa, ON, Canada
| | - Saurabh Malhotra
- Division of Cardiology, Cook County Health, Chicago, IL, USA
- Division of Cardiology, Rush Medical College, Chicago, IL, USA
| | - Ronny Buechel
- Department of Nuclear Medicine, Cardiac Imaging, University Hospital Zurich, Zurich, Switzerland
| | - Diwakar Jain
- Division of Cardiology and Nuclear Medicine, New York Medical College/Westchester Medical Center, Hawthorne, NY, USA
| | - Girish Dwivedi
- Fiona Stanley Hospital, Murdoch, WA, Australia
- Harry Perkins Institute of Medical Research, Murdoch, WA, Australia
- The University of Western Australia, Crawley, WA, Australia
| | - Mouaz H Al-Mallah
- Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, 6565 Fannin Street, Smith-19, Houston, TX, 77030, USA.
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Ödev K, Tunç R, Varol S, Aydemir H, Yılmaz PD, Korkmaz C. Thoracic Complications in Behçet's Disease: Imaging Findings. Can Respir J 2020; 2020:4649081. [PMID: 32566055 PMCID: PMC7275231 DOI: 10.1155/2020/4649081] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2020] [Accepted: 04/27/2020] [Indexed: 11/17/2022] Open
Abstract
Behçet's disease (BD) causes vascular inflammation and necrosis in a wide range of organs and tissues. In the thorax, it may cause vascular complications, affecting the aorta, brachiocephalic arteries, bronchial arteries, pulmonary arteries, pulmonary veins, capillaries, and mediastinal and thoracic inlet veins. In BD, chest radiograph is commonly used for the initial assessment of pulmonary symptoms and complications and for follow-up and establishment of the response to treatment. With the advancement of helical or multislice computed tomography (CT) technologies, such noninvasive imaging techniques have been employed for the diagnosis of vascular lesions, vascular complications, and pulmonary parenchymal manifestations of BD. CT scan (especially, CT angiography) has been used to determine the presence and severity of pulmonary complications without resorting to more invasive procedures, in conjunction with gadolinium-enhanced three-dimensional (3D) gradient-echo magnetic resonance (MR) imaging with the subtraction of arterial phase images. These radiologic methods have characteristics that are complementary to each other in diagnosis of the thoracic complications in BD. 3D ultrashort echo time (UTE) MR imaging (MRI) could potentially yield superior image quality for pulmonary vessels and lung parenchyma when compared with breath-hold 3D MR angiography.
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Affiliation(s)
- Kemal Ödev
- Department of Radiology, Konya Chamber of Commerce Karatay University, Faculty of Medicine, Konya, Turkey
| | - Recep Tunç
- Department of Rheumatology, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
| | - Salih Varol
- Department of Physiology, Konya Chamber of Commerce Karatay University, Faculty of Medicine, Konya, Turkey
| | - Harun Aydemir
- Department of Rheumatology, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
| | - Pınar Didem Yılmaz
- Department of Radiology, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
| | - Celalettin Korkmaz
- Department of Chest Clinic, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
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Navallas M, Inarejos Clemente EJ, Iglesias E, Rebollo-Polo M, Hernández JC, Navarro OM. Autoinflammatory diseases in childhood, part 2: polygenic syndromes. Pediatr Radiol 2020; 50:431-444. [PMID: 32065273 DOI: 10.1007/s00247-019-04544-9] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2019] [Revised: 07/29/2019] [Accepted: 09/20/2019] [Indexed: 02/08/2023]
Abstract
Autoinflammatory diseases are a family of disorders characterized by aberrant stimulation of inflammatory pathways without involvement of antigen-directed autoimmunity. They can be further divided in monogenic and polygenic types. Those without an identified genetic mutation are known as polygenic and include systemic-onset juvenile idiopathic arthritis, idiopathic recurrent acute pericarditis, Behçet syndrome, chronic recurrent multifocal osteomyelitis and inflammatory bowel disease among others. Autoinflammatory diseases are characterized by recurrent flares or persistent systemic inflammation and fever, as well as lymphadenopathy and cutaneous, abdominal, thoracic and articular symptoms. Although these syndromes can mimic infections clinically, the inflammatory lesions in autoinflammatory disorders are aseptic. However, because of their infrequency, varied and nonspecific presentation, and the new genetic identification, diagnosis is usually delayed. In this article, which is Part 2 of a two-part series, the authors review the main polygenic autoinflammatory diseases that can be seen in childhood, with special emphasis wherever applicable on imaging features that may help establish the correct diagnosis. However, the major role of imaging is to delineate organ involvement and disease extent.
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Affiliation(s)
- María Navallas
- Department of Radiology, Hospital Sant Joan de Déu, Passeig Sant Joan de Déu, 2, 08950 Esplugues de Llobregat, Barcelona, Spain. .,Department of Medical Imaging, University of Toronto, Toronto, ON, Canada. .,Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Canada.
| | - Emilio J Inarejos Clemente
- Department of Radiology, Hospital Sant Joan de Déu, Passeig Sant Joan de Déu, 2, 08950 Esplugues de Llobregat, Barcelona, Spain
| | | | - Mónica Rebollo-Polo
- Department of Radiology, Hospital Sant Joan de Déu, Passeig Sant Joan de Déu, 2, 08950 Esplugues de Llobregat, Barcelona, Spain
| | | | - Oscar M Navarro
- Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.,Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Canada
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Watanabe K, Tanida S, Inoue N, Kunisaki R, Kobayashi K, Nagahori M, Arai K, Uchino M, Koganei K, Kobayashi T, Takeno M, Ueno F, Matsumoto T, Mizuki N, Suzuki Y, Hisamatsu T. Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet's disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants. J Gastroenterol 2020; 55:679-700. [PMID: 32377946 PMCID: PMC7297851 DOI: 10.1007/s00535-020-01690-y] [Citation(s) in RCA: 48] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2020] [Accepted: 04/08/2020] [Indexed: 02/04/2023]
Abstract
Behçet's disease (BD) is an intractable systemic inflammatory disease characterized by four main symptoms: oral and genital ulcers and ocular and cutaneous involvement. The Japanese diagnostic criteria of BD classify intestinal BD as a specific disease type. Volcano-shaped ulcers in the ileocecum are a typical finding of intestinal BD, and punched-out ulcers can be observed in the intestine or esophagus. Tumor necrosis factor inhibitors were first approved for the treatment of intestinal BD in Japan and have been used as standard therapy. In 2007 and 2014, the Japan consensus statement for the diagnosis and management of intestinal BD was established. Recently, evidence-based JSBD (Japanese Society for BD) Clinical Practice Guidelines for BD (Japanese edition) were published, and the section on intestinal BD was planned to be published in English. Twenty-eight important clinical questions (CQs) for diagnosis (CQs 1-6), prognosis (CQ 7), monitoring and treatment goals (CQs 8-11), medical management and general statement (CQs 12-13), medical treatment (CQs 14-22), and surgical treatment (CQs 23-25) of BD and some specific situations (CQs 26-28) were selected as unified consensus by the members of committee. The statements and comments were made following a search of published scientific evidence. Subsequently, the levels of recommendation were evaluated based on clinical practice guidelines in the Medical Information Network Distribution Service. The degree of agreement was calculated using anonymous voting. We also determined algorithms for diagnostic and therapeutic approaches for intestinal BD. The present guidelines will facilitate decision making in clinical practice.
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Affiliation(s)
- Kenji Watanabe
- Department of Intestinal Inflammation Research, Hyogo College of Medicine, Hyogo, Japan
| | - Satoshi Tanida
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan
| | - Nagamu Inoue
- Centers for Preventive Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Reiko Kunisaki
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Kanagawa, Japan
| | - Kiyonori Kobayashi
- Research and Development Center for New Medical Frontiers, Kitasato University, School of Medicine, Kanagawa, Japan
| | - Masakazu Nagahori
- Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University, Tokyo, Japan
| | - Katsuhiro Arai
- Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan
| | - Motoi Uchino
- Department of Inflammatory Bowel Disease, Division of Surgery, Hyogo College of Medicine, Hyogo, Japan
| | - Kazutaka Koganei
- Department of Inflammatory Bowel Disease, Yokohama Municipal Citizen’s Hospital, Kanagawa, Japan
| | - Taku Kobayashi
- Center for Advanced IBD Research and Treatment, Kitasato University Kitasato Institute Hospital, Tokyo, Japan
| | - Mitsuhiro Takeno
- Department of Allergy and Rheumatology, Nippon Medical School Musashi Kosugi Hospital, Kanagawa, Japan
| | - Fumiaki Ueno
- Center for Gastroenterology and Inflammatory Bowel Disease, Ofuna Chuo Hospital, Kanazawa, Japan
| | - Takayuki Matsumoto
- Division of Gastroenterology, Department of Medicine, Iwate Medical University, Iwate, Japan
| | - Nobuhisa Mizuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Kanagawa, Japan
| | - Yasuo Suzuki
- Inflammatory Bowel Disease Center, Toho University Sakura Medical Center, Chiba, Japan
| | - Tadakazu Hisamatsu
- Department of Gastroenterology and Hepatology, Kyorin University School of Medicine, Tokyo, Japan
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Anand P, Chwalisz BK. Inflammatory Disorders of the Skull Base: a Review. Curr Neurol Neurosci Rep 2019; 19:96. [DOI: 10.1007/s11910-019-1016-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
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Soun JE, Song JW, Romero JM, Schaefer PW. Central Nervous System Vasculopathies. Radiol Clin North Am 2019; 57:1117-1131. [DOI: 10.1016/j.rcl.2019.07.005] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
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Rashaideh MA, Janho KE, Jalokh M, Ajarmeh ES, As'ad M. Ruptured Suprarenal Abdominal Aortic Pseudoaneurysm with Superior Mesenteric and Celiac Arteries Occlusion, Revealing Behçet's Disease: A Case Report. Vasc Specialist Int 2019; 35:160-164. [PMID: 31620402 PMCID: PMC6774426 DOI: 10.5758/vsi.2019.35.3.160] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2019] [Revised: 07/21/2019] [Accepted: 07/31/2019] [Indexed: 12/04/2022] Open
Abstract
Behçet’s disease (BD) is a multisystemic, chronic autoimmune inflammatory vasculitic disease with an unknown etiology. Although the literature reports that vascular involvement occurs in 7% to 38% of all BD cases, the arteries are rarely involved; however, arterial involvement is usually associated with significant mortality and morbidity. We report the case of a young female patient who presented to the emergency department with severe abdominal pain and a history of weight loss. The patient was evaluated using computed tomography angiography, which revealed a ruptured suprarenal aortic pseudoaneurysm with occlusion of both the superior mesenteric and celiac arteries. Urgent surgery was performed with aortic repair with an interposition graft and superior mesenteric artery embolectomy. The patient’s clinical history and radiological imaging findings were strongly suggestive of the diagnosis of BD with vascular involvement.
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Affiliation(s)
- Mohammed A Rashaideh
- Vascular Surgery Department, King Hussin Medical Center, Royal Medical Services, Amman, Jordan
| | - Kristi E Janho
- Vascular Surgery Department, King Hussin Medical Center, Royal Medical Services, Amman, Jordan
| | - Muhannad Jalokh
- Vascular Surgery Department, King Hussin Medical Center, Royal Medical Services, Amman, Jordan
| | - Eyad S Ajarmeh
- Vascular Surgery Department, King Hussin Medical Center, Royal Medical Services, Amman, Jordan
| | - Mohammed As'ad
- Vascular Surgery Department, King Hussin Medical Center, Royal Medical Services, Amman, Jordan
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Leitman EM, McDermott S. Pulmonary arteries: imaging of pulmonary embolism and beyond. Cardiovasc Diagn Ther 2019; 9:S37-S58. [PMID: 31559153 DOI: 10.21037/cdt.2018.08.05] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
The pulmonary arteries are not just affected by thrombus. Various acquired and congenital conditions can also affect the pulmonary arteries. In this review we discuss cross sectional imaging modalities utilized for the imaging of the pulmonary arteries. Acquired pulmonary artery entities, including pulmonary artery sarcoma (PAS), vasculitis, aneurysm, and arteriovenous malformations, and congenital anomalies in adults, including proximal interruption of the pulmonary artery, pulmonary sling, pulmonary artery stenosis, and idiopathic dilatation of the pulmonary trunk, are also discussed. An awareness of these entities and their imaging findings is important for radiologists interpreting chest imaging.
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Affiliation(s)
| | - Shaunagh McDermott
- Division of Thoracic Imaging and Intervention, Massachusetts General Hospital, Boston, Massachusetts, USA
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Neuro-Behçet Disease in the Central University Hospital of Asturias. ACTA ACUST UNITED AC 2019; 17:32-36. [PMID: 31078455 DOI: 10.1016/j.reuma.2019.03.005] [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: 01/04/2019] [Revised: 02/28/2019] [Accepted: 03/13/2019] [Indexed: 11/21/2022]
Abstract
OBJECTIVE To describe the frequency and profile of patients with neuro-Behçet's disease (NBD) at the Central University Hospital of Asturias between 1981 and June 2018. PATIENTS AND METHODS Retrospective study including epidemiological, clinical, neuroimaging, cerebrospinal fluid (CSF) study, histopathology, treatment and evolution characteristics. Clinical characteristics are differentiated between patients with Behçet without neurological affectation and NBD. RESULTS We found 10 cases of NBD (25.6%). The mean age was 29.7years, and it was more frequent in males. Sixty percent had parenchymal involvement. The non-parenchymal involvement included a case with cerebral venous thrombosis and two cases with isolated aseptic meningitis. Findings of vasculitis were found on cerebral magnetic resonance imaging, and alteration in the biochemistry of the CSF. One patient presented a striking motor disability. Ocular involvement was greater in the group of patients without neurological involvement (P=0.009). CONCLUSIONS NBD is a relatively frequent presentation, especially in males and in the parenchymal form. We did not find a systemic clinical marker of neurological involvement.
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Intestinal Behçet Disease: Evaluation With MR Enterography—A Case-Control Study. AJR Am J Roentgenol 2018; 211:767-775. [DOI: 10.2214/ajr.17.19174] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
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Abstract
Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Examining other organs usually affected by vasculitides (e.g., the skin and kidneys) and determining autoantibody levels are essential to a better management of the disease. A radiological study would also contribute to establishing a diagnosis. The lungs are commonly involved in small-vessel vasculitis, anti-glomerular basement membrane disease, and vasculitides associated with antineutrophil cytoplasmic antibodies. Associated life-threatening diffuse alveolar haemorrhages and irreversible damage to other organs-usually the kidneys-are severe complications that require early diagnosis. Vasculitides are rare diseases that affect multiple organs. An increasing number of treatments-including biological agent-based therapies-requiring cooperation between specialists and centers have become available in the recent years. In the same way, clinicians should be familiar with the complications associated with immunosuppressive therapies.
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Affiliation(s)
| | | | | | | | | | - Luis Valdés
- Interdisciplinary Research Group in Pneumology, Institute of Sanitary Research of Santiago de Compostela (IDIS), Santiago de Compostela, Spain
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Batra K, Chamarthy M, Chate RC, Jordan K, Kay FU. Pulmonary vasculitis: diagnosis and endovascular therapy. Cardiovasc Diagn Ther 2018; 8:297-315. [PMID: 30057877 DOI: 10.21037/cdt.2017.12.06] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
Pulmonary vasculitides are caused by a heterogeneous group of diseases with different clinical features and etiologies. Radiologic manifestations depend on the predominant type of vessel involved, which are grouped into large, medium, or small-sized vessels. Diagnosing pulmonary vasculitides is a challenging task, and radiologists play an important role in their management by providing supportive evidence for diagnosis and opportunities for minimally invasive therapy. This paper aims to present a practical approach for understanding the vasculitides that can affect the pulmonary vessels and lungs. We will describe and illustrate the main radiologic findings, discussing opportunities for minimally invasive treatment.
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Affiliation(s)
- Kiran Batra
- UT Southwestern Medical Center, Dallas, TX, USA
| | | | - Rodrigo Caruso Chate
- Hospital Israelita Albert Einstein and Instituto to Coração HCFMUSP, Sao Paulo, Brazil
| | - Kirk Jordan
- UT Southwestern Medical Center, Dallas, TX, USA
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Pu L, Li R, Xie J, Yang Y, Liu G, Wang Y, Li Y. Characteristic Echocardiographic Manifestations of Behçet's Disease. ULTRASOUND IN MEDICINE & BIOLOGY 2018; 44:825-830. [PMID: 29373154 DOI: 10.1016/j.ultrasmedbio.2017.12.010] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/11/2017] [Revised: 12/05/2017] [Accepted: 12/12/2017] [Indexed: 06/07/2023]
Abstract
The objective of this study was to determine the characteristic echocardiographic manifestations of Behçet's disease. Ninety-seven Behçet's patients admitted to our hospital from January 2002 to December 2016 were retrospectively analyzed. There were 63 patients (64.9%) with cardiac involvement; these included 47 men (74.6%) and 16 women (25.4%). Of these 63 patients, most (74.6%) exhibited valvular lesions, especially aortic regurgitation. The primary characteristic manifestations of valves were cusp prolapse with aneurysmal changes, vegetation-like lesions and echo-free spaces within the annulus. Second (30.0%) were aortic lesions, including aortic dilation or aneurysm formation, aortic pseudoaneurysm and coronary sinus aneurysm. Other manifestations, such as cardiac thrombosis, coronary arterial pseudoaneurysm, pulmonary aneurysm and pericardial effusion, were rare. Cardiac involvement is not uncommon in Behçet's patients, especially in males, and it is characterized by valvular disease, especially aortic regurgitation. The diagnosis of Behçet's disease should be considered when evaluating patients with these characteristic echocardiographic manifestations.
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Affiliation(s)
- Lihong Pu
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Rongjuan Li
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
| | - Jinjie Xie
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Ya Yang
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Guowen Liu
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Yueli Wang
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Yijia Li
- Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
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Imaging manifestations of Behcet's disease: Key considerations and major features. Eur J Radiol 2017; 98:214-225. [PMID: 29196115 DOI: 10.1016/j.ejrad.2017.11.012] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2017] [Revised: 09/26/2017] [Accepted: 11/17/2017] [Indexed: 01/16/2023]
Abstract
Behcet's disease is an autoimmune disease most commonly seen in the Middle East. Although primarily known with painful oral and genital ulcers, it can lead to vasculitis. Therefore, several associated complications such as thrombotic syndromes, aneurysmal arterial disease may arise. In many cases, it might be difficult to make the diagnosis purely based on clinical grounds; however, imaging plays an important role for both diagnosis and assessment of the disease's complications. We provide a comprehensive review of the most notable imaging findings of Behcet's disease.
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46
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Hur JH, Chun EJ, Kwag HJ, Yoo JY, Kim HY, Kim JJ, Lee KW. CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification. Korean J Radiol 2017; 18:786-798. [PMID: 28860896 PMCID: PMC5552462 DOI: 10.3348/kjr.2017.18.5.786] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2016] [Accepted: 02/14/2017] [Indexed: 12/26/2022] Open
Abstract
Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.
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Affiliation(s)
- Jee Hye Hur
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam 13620, Korea
| | - Eun Ju Chun
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam 13620, Korea
| | - Hyon Joo Kwag
- Department of Radiology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul 03181, Korea
| | - Jin Young Yoo
- Department of Radiology, Seoul National University Hospital, Seoul 03080, Korea
| | - Hae Young Kim
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam 13620, Korea
| | - Jeong Jae Kim
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam 13620, Korea
| | - Kyung Won Lee
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam 13620, Korea
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47
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Topcuoglu OM, Topcuoglu ED, Altay CM, Genc S. Imaging pearls of pediatric Behçet’s disease. Eur J Radiol 2017; 94:115-124. [DOI: 10.1016/j.ejrad.2017.06.016] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2017] [Revised: 06/18/2017] [Accepted: 06/21/2017] [Indexed: 12/30/2022]
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Cinar M, Yilmaz S, Akay S, Bozlar U, Dinc A. Clinical course of Behcet's disease in a patient with delayed diagnosis and radiological follow-up of the thrombi with computed tomography angiography: a five-year follow-up under immunosuppressive treatment. REVISTA BRASILEIRA DE REUMATOLOGIA 2017; 57:264-269. [PMID: 28535899 DOI: 10.1016/j.rbre.2013.08.004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2013] [Accepted: 08/12/2013] [Indexed: 06/07/2023] Open
Affiliation(s)
- Muhammet Cinar
- Gulhane Military Medical Academy School of Medicine, Division of Rheumatology, Ankara, Turkey.
| | - Sedat Yilmaz
- Gulhane Military Medical Academy School of Medicine, Division of Rheumatology, Ankara, Turkey
| | - Sinan Akay
- Gulhane Military Medical Academy School of Medicine, Department of Radiology, Ankara, Turkey
| | - Ugur Bozlar
- Gulhane Military Medical Academy School of Medicine, Department of Radiology, Ankara, Turkey
| | - Ayhan Dinc
- Gulhane Military Medical Academy School of Medicine, Division of Rheumatology, Ankara, Turkey
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Evolução clínica da doença de Behçet em paciente com atraso do diagnóstico e seguimento radiológico dos trombos com angiotomografia computadorizada: seguimento por 5 anos durante tratamento imunossupressor. REVISTA BRASILEIRA DE REUMATOLOGIA 2017. [DOI: 10.1016/j.rbr.2013.08.004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
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Mahmoud S, Ghosh S, Farver C, Lempel J, Azok J, Renapurkar RD. Pulmonary Vasculitis: Spectrum of Imaging Appearances. Radiol Clin North Am 2016; 54:1097-1118. [PMID: 27719978 DOI: 10.1016/j.rcl.2016.05.007] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
Pulmonary vasculitis is a relatively uncommon disorder, usually manifesting as part of systemic vasculitis. Imaging, specifically computed tomography, is often performed in the initial diagnostic workup. Although the findings in vasculitis can be nonspecific, they can provide important clues in the diagnosis, and guide the clinical team toward the right diagnosis. Radiologists must have knowledge of common and uncommon imaging findings in various vasculitides. Also, radiologists should be able to integrate the clinical presentation and laboratory test findings together with imaging features, so as to provide a meaningful differential diagnosis.
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Affiliation(s)
- Shamseldeen Mahmoud
- Section of Thoracic Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Subha Ghosh
- Section of Thoracic Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Carol Farver
- Department of Pathology, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Jason Lempel
- Section of Thoracic Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Joseph Azok
- Section of Thoracic Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Rahul D Renapurkar
- Section of Thoracic Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA; Section of Cardiovascular Imaging, Imaging Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
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