Anca-associated vasculitis - Open Access .click

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Current Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation

Life
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (ANCA-vasculitis) is an autoimmune disease characterized by inflammation and necrosis of small or medium vessels.
Konstantinos Drouzas +3 more
doaj +1 more source

Severe ADEM‐Like Neuroinflammatory Disease and Cerebrovascular Fragility With Recurrent Pseudoaneurysms and Moyamoya in a Familial Germline CBL Mutation: Expanding the Clinical Phenotype

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Heterozygous germline variants in CBL disrupt its function as a negative regulator of the Ras/MAPK pathway, classically predisposing to Juvenile myelomonocytic leukemia (JMML) and moyamoya. We describe two affected siblings carrying a paternally inherited CBL variant (c.1210 T> C, p.
Michal Bar‐Hakim +12 more
wiley +1 more source

Chronic Rhinosinusitis and Nasal Polyps in Eosinophilic Granulomatosis With Polyangiitis: A Scoping Review

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis (CRS) is one of the most common manifestations of eosinophilic granulomatosis with polyangiitis (EGPA), often preceding the diagnosis of systemic vasculitis by several years. The sinonasal presentation of EGPA typically resembles CRS with nasal polyps (CRSwNP), making it clinically indistinguishable from those
Alisha Sharma +12 more
wiley +1 more source

The immunopathology of ANCA-associated vasculitis [PDF]

Seminars in Immunopathology, 2014
The small-vessel vasculitides are a group of disorders characterised by variable patterns of small blood vessel inflammation producing a markedly heterogeneous clinical phenotype. While any vessel in any organ may be involved, distinct but often overlapping sets of clinical features have allowed the description of three subtypes associated with the ...
McKinney, E.F +3 more
openaire +3 more sources

A case of COVID‐19‐associated C‐ANCA vasculitis, was successfully treated with rituximab therapy

Clinical Case Reports
Key Clinical Message Both C‐anti‐neutrophil cytoplasmic antibody (ANCA) and P‐ANCA vasculitis were reported to be associated with COVID‐19 infection. The ideal management of COVID‐19‐associated ANCA vasculitis is unclear, as the experiences were limited ...
Michael Filoramo +5 more
doaj +1 more source

Effective Performance of the 2022 American College of Rheumatology/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study

Arthritis &Rheumatology, EarlyView.
Objective To assess the 2022 American College of Rheumatology (ACR)/EULAR classification criteria for antineutrophil cytoplasmic antibody–associated vasculitis (AAV) in children with chronic small‐to‐medium vessel vasculitis. Methods A cohort of 574 patients, identified by physician's diagnosis (MD‐diagnosis) in A Registry of Childhood Vasculitis, was ...
David A. Cabral +41 more
wiley +1 more source

Complement in ANCA-Associated Vasculitis

Seminars in Nephrology, 2013
Antineutrophil cytoplasmic autoantibodies (ANCA) are the likely cause for necrotizing small vessel vasculitis and crescentic glomerulonephritis. Unlike other forms of crescentic glomerulonephritis induced by immune complexes or anti-glomerular basement membrane (anti-GBM) antibodies that have conspicuous vessel wall immunoglobulin and complement, there
J, Charles Jennette, Hong, Xiao, Peiqi, Hu +2 more
openaire +3 more sources

Higher Complement C4 Gene Copy Number Constitutes a Shared Genetic Risk Factor for Giant Cell Arteritis and IgA Vasculitis

Arthritis &Rheumatology, EarlyView.
Objective Low copy number (CN) of complement C4 isoforms and high CN of retroviral HERV‐K elements are known risk factors for many immune‐mediated inflammatory diseases (IMIDs), often showing sex‐biased effects. Here, we assessed whether CN variation within the C4 gene contributes to giant cell arteritis (GCA) and IgA vasculitis (IgAV), two complex ...
Laura Martínez‐Gutiérrez +295 more
wiley +1 more source

Outcomes in progressive pulmonary fibrosis in systemic autoimmune rheumatic diseases: real‐world data from the ILD‐PRO Registry

Arthritis &Rheumatology, Accepted Article.
Objective We evaluated baseline characteristics, treatment patterns, and outcomes in patients with systemic autoimmune rheumatic disease‐associated progressive pulmonary fibrosis (SARD‐PPF) and evaluated whether outcomes differed by SARD subtype. Methods The ILD‐PRO Registry is a prospective multicenter US registry of patients with PPF.
Sonali J Bracken +7 more
wiley +1 more source

Targeting neutrophil extracellular traps in metabolic and immune niche: Nanomaterials for diabetes tissue regeneration

BMEMat, EarlyView.
The effects of NETs on regeneration of various diabetic tissues, and strategies targeting NETs for diabetes tissue regeneration. In the diabetic environment, NETs undergo complex metabolic and immune reprogramming, leading to dynamic changes in antibacterial and proinflammatory functions, and affecting regeneration of multiple systemic tissues.
Xinyi Jiang +6 more
wiley +1 more source

vasculitis
anca
medicine

antineutrophil cytoplasmic antibody
immunologic diseases
systemic sclerosis

allergy
systemic vasculitis
case report