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

Am J Kidney Dis. 1999 Apr;33(4):e3. doi: 10.1016/s0272-6386(99)70244-1.

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.

Publication types

  • Case Reports

MeSH terms

  • 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*

Substances

  • Antibodies
  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase