Bronchiolitis obliterans organising pneumonia and primary biliary cirrhosis-like lung involvement in a patient with primary biliary cirrhosis

Clin Rheumatol. 1998;17(3):246-9. doi: 10.1007/BF01451058.


A 55-year-old woman with a 6-year history of primary biliary cirrhosis presented with an acute onset of fever, dyspnoea, crackles over both lower lung fields, and diffuse interstitial and bibasilar patchy pulmonary opacities. After exclusion of an infectious aetiology, an open lung biopsy was performed which revealed two histopathological features: (1) bronchiolitis obliterans organising pneumonia and (2) lympho-histiocytic interstitial pneumonitis and destructive bronchiolitis. Treatment response to corticosteroids and azathioprine followed a bimodal pattern with immediate resolution of her initial presenting symptoms and late resolution of residual gas exchange defects.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Antimetabolites / therapeutic use
  • Azathioprine / therapeutic use
  • Biopsy, Needle
  • Bronchoalveolar Lavage
  • Cryptogenic Organizing Pneumonia / complications*
  • Cryptogenic Organizing Pneumonia / diagnosis
  • Cryptogenic Organizing Pneumonia / drug therapy
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Humans
  • Liver Cirrhosis, Biliary / complications*
  • Liver Cirrhosis, Biliary / diagnosis
  • Lung / pathology
  • Lung Diseases, Interstitial / complications*
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / drug therapy
  • Middle Aged
  • Respiratory Function Tests


  • Adrenal Cortex Hormones
  • Antimetabolites
  • Azathioprine