Korean J Med.  2009 Apr;76(Suppl 1):S154-S157.

ANCA-related crescentic glomerulonephritis in a patient with systemic sclerosis

• Kwon JS
• Shin YK
• Kwak JH
• Park SH
• Kim SK
• Choe JY

Abstract

The renal manifestations of systemic sclerosis include proteinuria, hypertension, azotemia, and renal crisis. Two types of scleroderma renal crisis (SRC) are recognized. Typical SRC is a syndrome consisting of acute-onset malignant hypertension accompanied by rapidly progressive renal failure, hypertensive retinopathy, and elevated plasma renin activity. The other type is normotensive renal failure, which is generally accompanied by antineutrophil cytoplasmic autoantibody (ANCA)-positive crescentic glomerulonephritis. A 51-year-old woman with scleroderma without marked dermatological change developed ANCA-related renal failure. She had neither malignant hypertension nor an elevated plasma rennin concentration. Renal biopsy showed crescentic glomerulonephritis (pauci-immune type), and the myeloperoxidase-specific ANCA (MPO-ANCA) titer was elevated at 1015 AAU. She was cured using steroid pulse therapy, combined with an angiotensin-converting-enzyme inhibitor and angiotensin-II receptor blocker

Keyword

Systemic sclerosis; ANCA; Crescentic glomerulonephritis; Renal crisis; Pulse therapy

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Azotemia
Biopsy
Chymosin
Cytoplasm
Female
Glomerulonephritis
Humans
Hypertension
Hypertension, Malignant
Hypertensive Retinopathy
Isonipecotic Acids
Middle Aged
Plasma
Proteinuria
Renal Insufficiency
Renin
Scleroderma, Systemic
Antibodies, Antineutrophil Cytoplasmic
Chymosin
Isonipecotic Acids
Renin