Granulomatosis with polyangiitis

被引：7

作者：

Kerstein, Anja ^{[1
,2
]}

Holl-Ulrich, Konstanze ^{[3
,4
]}

Mueller, Antje ^{[5
,6
]}

Riemekasten, Gabriela ^{[6
,7
]}

Lamprecht, Peter ^{[6
,7
]}

机构：

[1] Univ Lubeck, Klin Rheumatol, Lubeck, Germany

[2] Univ Lubeck, Vaskulitis Zentrum, Lubeck, Germany

[3] Univ Klinikums Schleswig Holstein, Konsultat & Referenzzentrums Vaskulitis Diagnost, Pathol, Campus Lubeck, Lubeck, Germany

[4] Forschungszentrums Borstel, Leibniz Zentrum Med & Biowissensch, Sulfeld, Germany

[5] Univ Lubeck, Klin Rheumatol, Forschungslab, Lubeck, Germany

[6] Univ Lubeck, Vaskulitis Zentrum, Ratzeburger Allee 160, D-23538 Lubeck, Germany

[7] Univ Lubeck, Klin Rheumatol, Ratzeburger Allee 160, D-23538 Lubeck, Germany

来源：

DEUTSCHE MEDIZINISCHE WOCHENSCHRIFT | 2017年 / 142卷 / 01期

关键词：

ANCA-ASSOCIATED VASCULITIS; ANTIBODY-ASSOCIATED VASCULITIS; SMALL-VESSEL VASCULITIS; TERM-FOLLOW-UP; WEGENERS-GRANULOMATOSIS; SYSTEMIC VASCULITIS; CELLS; GLOMERULONEPHRITIS; PATHOGENESIS; ASSOCIATION;

D O I：

10.1055/s-0042-111610

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Granulomatosis with polyangiitis (GPA) is a potentially lifethreatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.

引用

页码：24 / 31

页数：8

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