Nitta Kosaku
   Department   Other, Other
   Position  
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