MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease?

Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, protei...

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Bibliographic Details
Published inAmerican journal of kidney diseases Vol. 33; no. 4; p. e3
Main Authors Anders, H J, Wiebecke, B, Haedecke, C, Sanden, S, Combe, C, Schlöndorff, D
Format Journal Article
LanguageEnglish
Published United States 01.04.1999
Subjects
Aged
Antibodies - analysis
Antibodies, Antineutrophil Cytoplasmic - analysis
Fatal Outcome
Glomerulonephritis - complications
Glomerulonephritis - immunology
Glomerulonephritis - pathology
Humans
Kidney Glomerulus - pathology
Male
Peroxidase - immunology
Renal Insufficiency - etiology
Scleroderma, Systemic - complications
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Summary:Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from Japan, normotensive MPO-ANCA-positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.
ISSN:1523-6838