Diffuse fibrosing alveolitis and primary biliary cirrhosis

Diffuse fibrosing alveolitis (DFA) is a disease with progressive, diffuse fibrosis in the interstitial tissue of the pulmonary parenchyma. As a rule, diffuse fibrosing alveolitis is limited to the lungs alone but it may occur as a partial manifestation of a systemic disease. A case report is presented here where diffuse fibrosing alveolitis was encountered in a patient with primary biliary cirrhosis. The pulmonary symptoms dominated the clinical picture and investigation revealed the autoimmune condition.