A brief review of literature is made on the problem of modern classification, diagnosis and treatment of systemic vasculitis. The authors present a clinical case of a 40-YO male, in whom the disease started with focal neurological signs: epileptic seizures, disorder of consciousness, light euphoria, nasolabial fold insufficiency and left upper extremity hypoesthesia. Initial examination revealed upper sagittal sinus together with right lateral sinus thrombosis with forming of bilateral frontal lobe's hemorrhages; development of stable thrombocytopenia and platelet antibodies in blood species as well as hyperhomocysteinemia and a MTHFR gene mutation. Further, the patient was followed-up by the hematologist and treated by steroids. Systemic vasculitis of granulomatosis with polyangiitis (Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) were late diagnosed in this patient, and coexistence of two systemic vasculitis predetermined extremely severe course of the disease with fatal outcome in nearly 5 years after the disease started, and after steroid and rituximab treatment. It is concluded that the diagnosis of cerebral venous thrombosis in a young patient should exclude systemic vasculitis as one of its possible causes.
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http://dx.doi.org/10.17116/jnevro201611612230-35 | DOI Listing |
Rheumatology (Oxford)
January 2025
Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Objectives: To clarify the differences in clinical phenotypes, therapeutic patterns, and outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) across geographic regions using a multinational cohort.
Methods: Data were collected from patients with newly diagnosed or relapsing GPA or MPA in Europe, Japan, and the United States (US) from January to July 2020. The composite outcome of kidney failure and/or death within 52 weeks after treatment was evaluated, and the hazard ratios across the regions were estimated using the Cox proportional hazard model.
Cardiol Young
January 2025
Pediatric Cardiology, Rady Children's Hospital, University of California, San Diego, USA.
A 16-year-old male with newly diagnosed granulomatosis with polyangiitis presented to the emergency room with chest pain. He was found to have a myocardial infarction involving the right coronary artery and the left circumflex artery. He underwent mechanical thrombectomy and stent placement without significant sequelae.
View Article and Find Full Text PDFKorean J Intern Med
January 2025
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Background/aims: This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet's disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV).
Methods: We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis.
Cureus
December 2024
Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Kuala Lumpur, MYS.
Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis.
View Article and Find Full Text PDFJ Med Cases
January 2025
Department of Clinical Sciences, Malmo, Lund University, Malmo, Sweden.
Immunocompromised patients, especially those receiving B-cell depleting therapies, are at risk for developing atypical presentation with regard to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, with the potential for diagnostic delay and adverse outcomes if such delay occurs. A 66-year-old female with history of granulomatosis with polyangiitis (GPA) with previous pulmonary involvement, treated with rituximab and low-dose prednisolone, presented with prolonged fever and cough after having been treated at home for a mild SARS-CoV-2 infection in early July 2023. The patient had a prolonged course over several months with constitutional symptoms such as fever, cough and malaise.
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