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Deprecated: Implicit conversion from float 253.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 CEN+Case+Rep 2017 ; 6 (1): 39-45 Nephropedia Template TP
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A case of PR3?ANCA-positive anti-GBM disease associated with intrarenal arteritis and thrombotic microangiopathy #MMPMID28509125
Manabe S; Banno M; Nakano M; Fujii T; Kakuta Y; Nitta K; Hatano M
CEN Case Rep 2017[May]; 6 (1): 39-45 PMID28509125show ga
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.