Fibrosis, pulmonary

Scarring throughout the lungs which can be caused by many conditions such as, sarcoidosis, hypersensitivity pneumonitis, asbestosis, and certain medications. Chronic pulmonary inflammation and fibrosis can also occur without an identifiable cause, in which case it is referred to as idiopathic pulmonary fibrosis. Symptoms of pulmonary fibrosis include shortness of breath, coughing and diminished exercise tolerance. The treatment involves corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system. Interferon gamma-1b treatment for 12 months was reported to improve the status of idiopathic pulmonary fibrosis patients who did not respond to corticosteroid therapy, according to a report in The New England Journal of Medicine (Ziesche R et al. N Engl J Med 1999;341:1264-1269; and an accompanying editorial by du Bois RM N Engl J Med 1999;341:1302-1304.). The goal of treatment is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced with this condition. Pulmonary fibrosis can cause hypoxia (decreased oxygen in the blood) and this can lead to elevated pressure in the pulmonary artery (pulmonary hypertension) and this can in turn lead to failure of the right ventricle of the heart. Therefore, patients are frequently also treated with supplemental oxygen to prevent pulmonary hypertension.