A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA)

• Published in: Nihon Rinshō Menʾeki Gakkai kaishi Vol. 22; no. 2; pp. 86 - 92
• Main Authors: Harashima, S, Yoshizawa, S, Horiuchi, T, Nakashima, H, Niho, Y, Kusaba, T, Hayashida, I, Shinozaki, M, Katafuchi, R, Hirakata, H
• Format: Journal Article
• Language: Japanese
• Published: Japan 01.04.1999
• Subjects: Antibodies, Antineutrophil Cytoplasmic - blood; Biomarkers - blood; Female; Glomerulonephritis - complications; Glomerulonephritis - diagnosis; Humans; Middle Aged; Polyarteritis Nodosa - diagnosis; Scleroderma, Systemic - complications; Scleroderma, Systemic - diagnosis; Syndrome
• ISSN: 0911-4300; 1349-7413

A 50-year-old female with systemic sclerosis (SSc) developed rapidly progressive renal insufficiency. Laboratory findings showed rapid elevation of serum creatinine level (3.8 mg/dl) and a high titer of perinuclear-antineutrophil cytoplasmic antibody (p-ANCA) (504 EU/ml). Renal pathology revealed crescentic glomerulonephritis (CrGN) without mucoid intimal proliferation of the interlobal arteries and fibrinoid necrosis of the afferent arterioles, Immunofluorescent micrography showed focal segmental granular deposition of IgG and C 3 in the mesangium and along the capillary loop and was in agreement with pauci-immune type. Recently, a subtype of renal involvement in SSc that is associated exclusively with normotensive renal failure and recognizable by p-ANCA is suggested. On the other hand, SSc with p-ANCA-positive glomerulonephritis as this case can be considered to be overlap syndrome of SSc and microscopic polyangitis nodosa (microscopic PN) because in microscopic PN, p-ANCA is detected at the range of 50% to 80% and renal pathology reveals necrotizing glomerulonephritis. In this point, we may describe this case as a suggestive one about p-ANCA-positive glomerulonephritis.