An Autopsy Case of Myeloperoxidase-anti-neutrophil Cytoplasmic Antibody (MPO-ANCA)-associated Vasculitis Accompanied by Cryoglobulinemic Vasculitis Affecting the Kidneys, Skin, and Gastrointestinal Tract

被引：3

作者：

Hasegawa, Jumpei ^{[1
,2
]}

Wakai, Sachiko ^{[1
]}

Kono, Momoko ^{[1
]}

Imaizumi, Yusuke ^{[1
]}

Masuda, Sakiko ^{[3
]}

Ishizu, Akihiro ^{[3
]}

Honda, Kazuho ^{[4
]}

机构：

[1] Tokyo Metropolitan Hlth & Med Treatment Corp, Okubo Hosp, Dept Nephrol, Tokyo, Japan

[2] Tokyo Womens Med Univ, Dept Urol, Tokyo, Japan

[3] Hokkaido Univ, Fac Hlth Sci, Sapporo, Hokkaido, Japan

[4] Showa Univ, Sch Med, Dept Anat, Tokyo, Japan

来源：

INTERNAL MEDICINE | 2018年 / 57卷 / 18期

基金：

日本学术振兴会;

关键词：

ANCA; anti-neutrophil cytoplasmic antibody; cryoglobulin; vasculitis; neutrophil extracellular traps; NETs; SMALL-VESSEL VASCULITIS; GLOMERULONEPHRITIS; AUTOIMMUNE; ANCA;

D O I：

10.2169/internalmedicine.0720-17

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) and cryoglobulinemic vasculitis (CV) rarely coexist. An 83-year-old woman was admitted with rapidly progressive renal failure, gastrointestinal hemorrhage and purpura with myeloperoxidase (MPO)-ANCA positivity and cryoglobulinemia. Despite intensive immunosuppressive treatment, she died of aspergillus pneumonia. Autopsy revealed necrotizing crescentic glomerulitis in the majority of the glomeruli, accompanied by partially membranoproliferative-like glomerular changes. Immunofluorescence staining revealed the presence of neutrophil extracellular trap (NET) formation in the glomeruli and cutaneous arteries. These pathological findings suggested that MPO-AAV and/or CV caused NET formation, leading to lethal systemic vasculitis.

引用

页码：2739 / 2745

页数：7

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