Advanced pulmonary sarcoidosis☆

Sarcoidosis affects the lungs in most patients. Manifestations of pulmonary sarcoidosis range from asymptomatic to death. Approximately a quarter of sarcoidosis patients develop chronic pulmonary disease. Advanced pulmonary sarcoidosis patients are those who have progressive disease and are at risk for significant morbidity and mortality. There are several features associated with advanced pulmonary disease: pulmonary fibrosis, pulmonary hypertension, chronic pulmonary inflammation, and/or complications of disease or therapy. Large retrospective studies have identified pulmonary fibrosis and pulmonary hypertension as the major causes of respiratory failure and death in pulmonary sarcoidosis. The high-resolution computer tomography scan (HRCT) and echocardiogram are key methods in screening for pulmonary hypertension and pulmonary fibrosis. Therapy for chronic pulmonary inflammation has been the major focus in chronic disease. However, treatment for pulmonary hypertension has been studied in sarcoidosis. To date, treatment studies for sarcoidosis associated progressive pulmonary fibrosis have been underpowered to demonstrate clear cut benefit of anti-fibrotic agents.