The term vasculitis is defined as inflammation of the blood vessel wall. Small-vessel vasculitides affect post-capillary venules, mostly in the skin. Defining the different types of small-vessel vasculitides is important as they may be associated with systemic manifestations, may require additional patient evaluation and may require more than only supportive care. Evaluation of a patient with suspected small-vessel vasculitis requires skin biopsy, evaluation of the extent of the disease, and an attempt to define an etiology. Management is based on providing supportive care, treating any underlying condition, avoiding any triggers, and treating based on severity of skin lesions and systemic involvement.
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http://dx.doi.org/10.1111/j.1529-8019.2012.01535.x | DOI Listing |
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de Coimbra, Coimbra, PRT.
Microscopic polyangiitis (MPA) is a rare, autoimmune, small-vessel vasculitis usually described with the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). It encompasses a broad spectrum of clinical features, including fatigue, weight loss, fever, arthralgia, skin lesions, and involvement of the lungs or kidneys. Ocular manifestations, however, are extremely rare.
View Article and Find Full Text PDFCureus
December 2024
Medicine, Faculdade de Medicina da Universidade do Porto (FMUP), Porto, PRT.
Anti-glomerular basement membrane disease is a rare small vessel vasculitis caused by the deposition of immunoglobulin G (IgG) autoantibodies in the basement membrane of glomerular capillaries and lung alveoli, leading to rapidly progressive renal failure and/or alveolar hemorrhage. We report the case of an 83-year-old female patient presenting with uremic symptoms, rapidly progressive kidney failure, and a high titer of anti-glomerular basement membrane antibodies. Given the urgent need for kidney replacement therapy, the substantial fibrosis and glomerular scarring observed in the kidney biopsy suggesting a chronic process, and the absence of pulmonary involvement, neither immunosuppressive treatment nor plasmapheresis was initiated, since a low likelihood of a favorable response to these interventions was expected.
View Article and Find Full Text PDFHum Immunol
January 2025
The Second Affiliated Hospital of Guangxi Medical University, Department of Nephrology, Nanning, Guangxi 530021, China. Electronic address:
Background: Microscopic polyangiitis (MPA) is a severe multisystem autoimmune disease featured by small-vessel vasculitis with few or no immune complex, also has a significant genetic predisposition. Growing evidence has confirmed that STAT4 gene is tightly associated with multiple autoimmune diseases, but its contribution to MPA onset is still elusive.
Objective: The aim was to investigated the association between STAT4 gene polymorphisms (rs7572482, rs7574865 and rs12991409) and MPA susceptibility in a Guangxi population of China.
Biomedicines
January 2025
Discipline of Clinical Laboratory and Food Safety, Faculty of Pharmacy, "Carol Davila" University of Medicine and Pharmacy, 6 Traian Vuia Street, 020945 Bucharest, Romania.
Immunoglobulin A (IgA) vasculitis (IgAV), classically known as Henoch-Schönlein purpura (HSP), is a type of nonthrombocytopenic small-vessel vasculitis. HSP is the most frequent kind of systemic vasculitis in children, characterized by purpura, arthritis or arthralgia, gastrointestinal pain, and kidney dysfunction. The aim of our research was to investigate and observe the clinical characteristics of children diagnosed with HSP and to explore the correlation between infectious diseases and HSP.
View Article and Find Full Text PDFNefrologia (Engl Ed)
January 2025
Unidad de Enfermedades Sistémicas Autoinmunes, Departamento de Medicina Interna, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is characterised by small vessel necrotising inflammatory vasculitis. Prior to immunosupressant therapy availability it usually led to a fatal outcome. Current treatment has changed ANCA-associated vasculitis into a condition with a significant response rate, although with a not negligible relapse occurrence and cumulative organ lesions, mostly due to drug-related toxicities.
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