Diseases of the hepatopulmonary axis

Hepatopulmonary syndrome is the most widely recognized of the processes associated with end-stage liver disease. Chronic liver dysfunction is associated with pulmonary manifestations due to alterations in the production or clearance of circulating cytokines and other mediators. Hepatopulmonary syndrome results in hypoxemia due to pulmonary vasodilatation with significant arteriovenous shunting and ventilation-perfusion mismatch. Hepatic hydrothorax may develop in patients with cirrhosis and ascites. Rarely, pulmonary hypertension occurs in the setting of portal hypertension. A second group of disorders may primarily affect the lungs and liver (the hepatopulmonary axis). Among these are the congenital conditions alpha(1)-antitrypsin deficiency and cystic fibrosis. Autoimmune liver disease may be associated with lymphocytic interstitial pneumonitis, fibrosing alveolitis, intrapulmonary granulomas, and bronchiolitis obliterans with organizing pneumonia. Sarcoidosis affects the lung and liver in up to 70% of patients. Medications such as amiodarone can result in a characteristic radiologic appearance of pulmonary and hepatic toxic effects. Knowledge of these associations will assist the radiologist in forming a meaningful differential diagnosis and may influence treatment decisions.