Antineutrophil Cytoplasmic Antibody-Positive Crescentic Glomerulonephritis in Scleroderma--A Different Kind of Renal Crisis

J Rheumatol. 2006 Sep;33(9):1886-8. Epub 2006 Jul 1.

Abstract

Renal disease remains a major source of morbidity and mortality in patients with scleroderma (systemic sclerosis, SSc). We describe the clinical course of 3 patients with diffuse cutaneous SSc presenting with renal disease subsequently found to have antibodies to myeloperoxidase (anti-MPO) and crescentic glomerulonephritis. The presence of antineutrophil cytoplasmic antibodies (ANCA) and anti-MPO defines a subset of patients with SSc who are susceptible to crescentic glomerulonephritis. These patients may present in a manner identical to scleroderma renal crisis, yet treatment requirements differ significantly. We suggest that the presence of ANCA be routinely evaluated when faced with renal failure in the setting of SSc.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Autoantibodies / blood*
  • Diagnosis, Differential
  • Female
  • Glomerulonephritis / blood*
  • Glomerulonephritis / etiology*
  • Humans
  • Male
  • Middle Aged
  • Peroxidase / immunology*
  • Renal Insufficiency / blood
  • Renal Insufficiency / etiology
  • Scleroderma, Systemic / blood*
  • Scleroderma, Systemic / complications*

Substances

  • Autoantibodies
  • Peroxidase