Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis

Clin Rheumatol. 2012 Aug;31(8):1273-7. doi: 10.1007/s10067-012-2010-8. Epub 2012 May 30.

Abstract

Pauci-immune necrotizing glomerulonephritis is a form of a rapidly progressive glomerulonephritis that is associated with the anti-neutrophil cytoplasmic antibodies (ANCA). ANCA-associated pauci-immune glomerulonephritis may be secondary to rheumatic diseases. We report on a patient with limited cutaneous systemic sclerosis who presented with subacute onset of nephritic syndrome and renal insufficiency. Her myeloperioxidase ANCA titer was elevated and renal biopsy confirmed pauci-immune crescentic glomerulonephritis, which was stabilized with high-dose glucocorticoid and cyclophosphamide. ANCA-associated necrotizing glomerulonephritis is rarely reported in systemic sclerosis and a systematic review is presented.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Antirheumatic Agents / administration & dosage
  • Azathioprine / administration & dosage
  • Biopsy
  • Cyclophosphamide / administration & dosage
  • Drug Therapy, Combination
  • Female
  • Glomerulonephritis / complications*
  • Glomerulonephritis / drug therapy
  • Glucocorticoids / administration & dosage
  • Humans
  • Middle Aged
  • Prednisolone / administration & dosage
  • Renal Insufficiency / etiology
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / drug therapy

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antirheumatic Agents
  • Glucocorticoids
  • Cyclophosphamide
  • Prednisolone
  • Azathioprine