Microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?

Am J Med Sci. 2001 Mar;321(3):201-2. doi: 10.1097/00000441-200103000-00009.


We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / analysis*
  • Female
  • Glomerulonephritis
  • Hematuria
  • Humans
  • Middle Aged
  • Proteinuria
  • Pulmonary Fibrosis / complications
  • Pulmonary Fibrosis / diagnosis*
  • Vasculitis / complications
  • Vasculitis / diagnosis*


  • Antibodies, Antineutrophil Cytoplasmic