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2017 ; 6
(1
): 39-45
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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
PMID28509125
show 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.