BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA AND PRIMARY BILIARY CIRRHOSIS-LIKE LUNG INVOLVEMENT IN A PATIENT WITH PRIMARY BILIARY-CIRRHOSIS

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 pulmo nary opacities. After exclusion of an infectious aetiology, an open lu ng biopsy was performed which revealed two histopathological features: (1) bronchiolitis obliterans organising pneumonia and (2) lympho-hist iocytic interstitial pneumonitis and destructive bronchiolitis. Treatm ent response to corticosteroids and azathioprine followed a bimodal pa ttern with immediate resolution of her initial presenting symptoms and late resolution of residual gas exchange defects.