If a causative agent can be identified (e.g., thermophilic actinomyces in hypersensitivity pneumonitis), cessation of exposure to that agent is imperative. Because the response to treatment among different ILDs is so variable, identification of treatable causes is essential. Glucocorticoids can be highly effective for eosinophilic pneumonias, COP, hypersensitivity pneumonitis (HP), acute radiation pneumonitis, and drug-induced ILD. Prednisone at 0.5-1.0 mg/kg per day is commonly given for 4-12 weeks, followed by a gradual tapering dose. On the other hand, glucocorticoids are usually not beneficial in IPF. Smoking cessation is essential, especially for smoking-related ILDs such as PLCH and respiratory bronchiolitis.

Supportive therapeutic measures include providing supplemental O₂ for pts with significant hypoxemia (Pa_O2<55 mmHg at rest and/or with exercise). Pulmonary rehabilitation is often beneficial. For younger pts with end-stage ILD, lung transplantation should be considered.