Microscopic polyangiitis associated with diffuse panbronchiolitis

Intern Med. 2004 Apr;43(4):331-5. doi: 10.2169/internalmedicine.43.331.

Abstract

There are several case reports of systemic vasculitis associated with chronic suppurative lung diseases. We describe a 46-year-old female, previously diagnosed as having diffuse panbronchiolitis (DPB), presenting with hemosputum and dyspnea. Her serum titer of MPO-ANCA was positive together with a high titer of BPI-ANCA. Chest X-ray and chest CT scan showed pulmonary hemorrhage, and the renal biopsy specimen revealed necrotizing, crescentic glomerulonephritis. She was diagnosed as having ANCA-associated vasculitis, and more specifically, microscopic polyangiitis accompanied by DPB. She was treated with methylprednisolone pulse therapy, followed by intravenous cyclophosphamide. This case suggested a possible association with chronic bacterial infection, which may play a role in the pathogenesis of ANCA-associated vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / analysis
  • Bronchiolitis / complications*
  • Bronchiolitis / diagnostic imaging
  • Bronchiolitis / immunology
  • Cholera Toxin
  • Female
  • Humans
  • Middle Aged
  • Peroxidase
  • Radiography
  • Vasculitis / complications*

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Cholera Toxin
  • Peroxidase