Renal-limited vasculitis with elevated levels of multiple antibodies
2017
Renal-limited
vasculitis(RLV) is a type of
anti-neutrophil cytoplasmic antibody(ANCA)-associated
vasculitisthat presents with
crescentic glomerulonephritiswith no other organ involvement. Although several studies reported patients with
crescentic glomerulonephritiswho were dual positive for
proteinase 3(PR3)-ANCA and
myeloperoxidase(MPO)-ANCA or ANCA and anti-
glomerular basement membrane(GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with
pauci-immunetype
crescentic glomerulonephritispositive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody.
Renal biopsyled to the definitive diagnosis of RLV with
pauci-immune-type
crescentic glomerulonephritis. Fluorescence immunostaining showed no linear deposition of IgG on GBM, indicating no involvement of anti-GBM associated diseases. Intensive therapy, including prednisolone, plasma exchange, and intravenous
cyclophosphamide, was effective. We report the case of triple-positive patient with
crescentic glomerulonephritis, who was successfully treated with glucocorticoid, plasma exchange, and
cyclophosphamide, suggesting that treatment for RLV in the patient with serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against
microscopic polyangiitis.
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