Renal-limited
vasculitis (RLV) is a type of
anti-neutrophil cytoplasmic antibody (
ANCA)-associated vasculitis that presents with crescentic
glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic
glomerulonephritis who 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-immune type crescentic
glomerulonephritis positive for MPO-
ANCA, PR3-ANCA, and
anti-GBM antibody. Renal biopsy led 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.