Definition of Pulmonary fibrosis

Pulmonary fibrosis: Scarring throughout the lungs which can be caused by many conditions such as, sarcoidosis, hypersensitivity pneumonitis, asbestosis, and certain medications. Pulmonary fibrosis can also occur without an identifiable cause, in which case it is referred to as idiopathic pulmonary fibrosis. Symptoms include shortness of breath, coughing and diminished exercise tolerance. Treatment involves the use of corticosteroids (such as prednisone) and/or other medications that suppress the body's immune system. Interferon gamma-1b treatment may help patients with idiopathic pulmonary fibrosis who do not respond to corticosteroid therapy. The goal of treatment is to decrease lung inflammation and subsequent scarring. Responses to treatment vary. Toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis are generally cared for by lung specialists.

Scarring of the alveoli reduces the ability of the lungs to transfer oxygen. The resulting lack of blood oxygen (hypoxemia) may increase the pressure in the arteries to the lungs, a situation known as pulmonary hypertension. This puts a strain on the right ventricle of the heart, which pumps blood to the lungs, and may lead to right heart failure. Patients are often treated with supplemental oxygen to resupply the blood with oxygen. As a consequence, breathlessness is reduced, the patient can be more active, and the severity of pulmonary hypertension may decrease.