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Interstitial lung diseases (ILDs) are a varied group of disorders characterized by inflammation and/or pulmonary fibrosis that impairs lung function and gas exchange. Known causes of ILD include connective tissue or systemic autoimmune rheumatic diseases, genetic abnormalities, pneumotoxic medications or treatments, infections, occupational exposure to materials such as asbestos, and smoking, but in many cases, the cause of ILD is unknown. Timely and accurate diagnosis is best achieved with a multidisciplinary assessment that involves clinical examination, thorough patient history, evaluation of environmental, occupational, or drug-induced exposures, imaging, pulmonary function testing, laboratory testing, and in some cases, lung biopsy.