Idiopathic pulmonary fibrosis

Scarring throughout the lungs without an identifiable cause. 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.

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chronic inflammation and progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting finally in death from oxygen lack or right heart failure. Sometimes it is a component of bronchiolitis obliterans with organizing pneumonia (see under bronchiolitis).The acute, rapidly fatal form is called acute interstitial pneumonia or Hamman-Rich syndrome. Called also chronic fibrous pneumonia, interstitial or usual interstitial pneumonia, diffuse interstitial pulmonary f., fibrosing alveolitis, and interstitial or interstitial pulmonary f.