title: MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease?
abstract: 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.
authors: Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany. hjanders@clinbio.med.uni-muenchen.de
authors: H J
authors: HJ
authors: Anders
authors: B
authors: B
authors: Wiebecke
authors: C
authors: C
authors: Haedecke
authors: S
authors: S
authors: Sanden
authors: C
authors: C
authors: Combe
authors: D
authors: D
authors: Schlöndorff
mesh_terms: D000368:Aged; D000906:Antibodies / Q000032:analysis; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D017809:Fatal Outcome; D005921:Glomerulonephritis / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D009195:Peroxidase / Q000276:immunology; D051437:Renal Insufficiency / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications*
chemical_list: D000906:Antibodies; D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase