ANCA-Associated Vasculitis: An Update

被引：95

作者：

Almaani, Salem ^{[1
]}

Fussner, Lynn A. ^{[2
]}

Brodsky, Sergey ^{[3
]}

Meara, Alexa S. ^{[4
]}

Jayne, David ^{[5
]}

机构：

[1] Ohio State Univ, Div Nephrol, Wexner Med Ctr, Columbus, OH 43201 USA

[2] Ohio State Univ, Div Pulm & Crit Care Med, Wexner Med Ctr, Columbus, OH 43201 USA

[3] Ohio State Univ, Dept Pathol, Wexner Med Ctr, Columbus, OH 43201 USA

[4] Ohio State Univ, Div Rheumatol, Wexner Med Ctr, Columbus, OH 43201 USA

[5] Univ Cambridge, Dept Med, Cambridge CB2 0QQ, England

来源：

JOURNAL OF CLINICAL MEDICINE | 2021年 / 10卷 / 07期

关键词：

ANCA vasculitis; review; vasculitis; crescentic glomerulonephritis; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; DAILY ORAL CYCLOPHOSPHAMIDE; CHURG-STRAUSS-SYNDROME; WEGENERS-GRANULOMATOSIS; MICROSCOPIC POLYANGIITIS; PLASMA-EXCHANGE; MAINTENANCE THERAPY; RANDOMIZED-TRIAL; RISK-FACTORS; REMISSION-MAINTENANCE;

D O I：

10.3390/jcm10071446

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.

引用

页数：22

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