マナベ　シユン   Manabe Shiyun   眞部　俊    所属   医学部 医学科（東京女子医科大学病院）    職種   講師
論文種別	原著
言語種別	英語
査読の有無	査読なし
表題	A case of PR3-ANCA-positive anti-GBM disease associated with intrarenal arteritis and thrombotic microangiopathy.
掲載誌名	正式名：CEN Case Reports 略　 称：CEN Case Rep ISSNコード：(2192-4449)2192-4449(Linking)
巻・号・頁	6(1),pp.39-45
著者・共著者	Manabe Shun, Banno Mayuko, Nakano Marie, Fujii Teruhiro, Kakuta Yukio, Nitta Kosaku, Hatano Michiyasu
発行年月	2017/05
概要	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