Granulomatosis with Polyangiitis and Microscopic Polyangiitis

被引：0

作者：

Klapa, Sebastian ^{[1
]}

Arnold, Sabrina ^{[1
]}

Lamprecht, Peter ^{[1
,2
]}

机构：

[1] Univ Lubeck, Dept Rheumatol & Clin Immunol, Lubeck, Germany

[2] Univ Lubeck, Dept Rheumatol, Ratzeburger Allee 160, D-23538 Lubeck, Germany

来源：

AKTUELLE RHEUMATOLOGIE | 2023年 / 48卷 / 01期

关键词：

ANCA; AAV; vasculitis; granulomatosis with polyangiitis; microscopic polyangiitis; RHEUMATOLOGY CLASSIFICATION CRITERIA; ANCA-ASSOCIATED VASCULITIS; 2022; AMERICAN-COLLEGE; TERM-FOLLOW-UP; WEGENERS-GRANULOMATOSIS; APOPTOTIC CELLS; CYCLOPHOSPHAMIDE; RITUXIMAB; REMISSION; DISEASE;

D O I：

10.1055/a-1963-7586

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two entities of ANCA-associated vasculitis (AAV). Both diseases are characterised by systemic necrotising small-vessel vasculitis, which can affect any organ. In GPA, extravascular necrotising granulomatous inflammation, usually affecting the respiratory tract, is found in addition. In the majority of cases, the clinical presentation is dominated by a pulmonary-renal syndrome with alveolar haemorrhage and rapidly progressive glomerulonephritis. Other organ involvement is found as well. In GPA, the upper respiratory tract is commonly affected. GPA is associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) and MPA with specificity for myeloperoxidase (MPO-ANCA). Immunosuppressive therapy depends on disease activity and the severity of organ involvement.

引用

页码：60 / 69

页数：10

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