眞部 俊
Department School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine Position Assistant Professor |
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Article types | Original article |
Language | English |
Peer review | Non peer reviewed |
Title | A case of PR3-ANCA-positive anti-GBM disease associated with intrarenal arteritis and thrombotic microangiopathy. |
Journal | Formal name:CEN Case Reports Abbreviation:CEN Case Rep ISSN code:(2192-4449)2192-4449(Linking) |
Volume, Issue, Page | 6(1),pp.39-45 |
Author and coauthor | Manabe Shun, Banno Mayuko, Nakano Marie, Fujii Teruhiro, Kakuta Yukio, Nitta Kosaku, Hatano Michiyasu |
Publication date | 2017/05 |
Summary | Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) is occasionally reported and termed "double positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA, and some of the MPO-ANCA-positive cases reveal intrarenal arteritis indicating an ANCA-associated renal lesion. In contrast, proteinase 3 (PR3)-ANCA-positive "double positive" disease had rarely been reported, and as far as we know, none of the cases showed intrarenal arteritis. Herein, we report a case of PR3-ANCA-positive "double positive" anti-GBM disease presenting with pulmonary-renal syndrome and hemolytic uremic syndrome. The kidney biopsy showed crescentic glomerulonephritis, intrarenal arteritis, and thrombotic microangiopathy. This case newly describes PR3-ANCA-associated intrarenal arteritis in "double positive" anti-GBM disease. |
DOI | 10.1007/s13730-016-0240-3 |
PMID | 28509125 |