Pulmonary manifestations of primary autoimmune hepatobiliary disease

The autoimmune hepatobiliary diseases are associated with a variety of pulmonary manifestations. There is increasing evidence of autoimmune pathophysiology occurring within the hepatopulmonary axis that may lead to clinically evident disease. Primary biliary cirrhosis (PBC) figures prominently among the primary autoimmune hepatobiliary processes with pulmonary involvement. PBC has been associated with subclinical alveolitis, sarcoidosis, bronchiolitis obliterans with organizing pneumonia, and lymphoid interstitial pneumonia, among others. Clinical associations have also been made between primary sclerosing cholangitis (PSC) and sarcoidosis, pulmonary fibrosis, or alveolar hemorrhage. Last, an evaluation of the current diagnostic criteria of autoimmune hepatitis has led to a reconsideration of its association with pulmonary fibrosis. Through a heightened clinical suspicion for these potential relationships, an improvement can be made in our comprehension of these autoimmune hepatobiliary processes and their impact on pulmonary physiology.