Advances in interstitial lung disorders.

Abstract

Interstitial lung disorders are a heterogeneous group of diseases that result in a similar clinical presentation and have similar physiologic consequences on lung function. Our current understanding of these disorders indicates that there is an inflammatory component of these diseases that is reversible and that precedes the development of interstitial pulmonary fibrosis, which is irreversible. Although conclusive clinical studies are still lacking, treatment of pulmonary disease in these patients is based on the concept that treatment of the inflammatory component of the disease with immunosuppressive agents will prevent or reduce the amount of pulmonary fibrosis that develops. Because of the significant side effects associated with immunosuppressive drugs, therapy should be used only when there is likely to be therapeutic benefit. The use of immunosuppressive agents is, therefore, indicated in selected groups of patients. If there is a known precipitating agent for the interstitial disorder, such as asbestos exposure, the primary therapy is to avoid further exposure to the agent. Sarcoidosis is one of the most common systemic disorders associated with interstitial lung disease, and in this disease, corticosteroids clearly are of benefit. In pulmonary sarcoidosis, patients who are symptomatic or patients who demonstrate progressive clinical deterioration of pulmonary function should be treated. Recent studies also suggest that patients with a high degree of pulmonary inflammation as demonstrated by a positive gallium scan and a high percentage (greater than 28%) of lymphocytes obtained on lung lavage may also benefit from corticosteroid therapy. Idiopathic pulmonary fibrosis is a progressive disease and is usually symptomatic at the time of presentation, so it is reasonable to give all patients a therapeutic trial with corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)