Renal-limited vasculitis with elevated levels of multiple antibodies

Abstract

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.

Keywords: Anti-GBM antibody; Case report; MPO-ANCA; PR3-ANCA.

Fig. 1

Computed tomography (CT) of the chest. A CT image shows a localized consolidation in right upper lung

Fig. 2

Renal biopsy specimen included 30 glomeruli, including 5 cellular crescents and 6 fibrocellular crescents. a Representative glomerulus containing cellular crescents with necrotizing lesion is shown (Periodic acid Schiff staining ×200). b Small artery exhibited granulomatous reaction with multinucleated giant cell formation (A arrow) and the destruction of elastic fibers (Elastica van Gieson staining ×200). c There was no staining of the IgG on immunofluorescence staining (×200)

Fig. 3

Clinical course. The y-axis of the left side shows the level of serum creatinine; the right side shows the titer of ANCA and anti-GBM antibody (aGBM Ab). After the treatment of intravenous high-dose methylprednisolone (mPSL) (days 2–4) followed by oral prednisolone (PSL), plasma exchange (PE: days 3,6,7,8,11), and intravenous high-dose cyclophosphamide therapy (IVCY: days 20,34), laboratory data were gradually improved. Bx, renal biopsy