Interstitial lung disease (ILD) is a heterogeneous group of lung conditions of both known and unknown etiology. There are more than 200 different diseases in the group, which broadly can be divided into infectious, occupational, iatrogenic, granulomatous, malignant, autoimmune/connective tissue disorder-related, and idiopathic categories (Table 1). The incidence and mortality from ILD seems to be increasing. Despite disparate etiologies, they often have similar clinical features, including dyspnea and hypoxemia, restrictive spirometry, depressed diffusion capacity, and a diffusely abnormal interstitium on lung imaging. The presentation of the idiopathic etiologies can vary from a slow, insidious presentation to subacute lung failure with a wide variety of expected responses to therapy (Table 2). Patients with ILD present a therapeutic conundrum for clinicians because these varied disorders with similar clinical presentations often require radically different therapies (e.g., antimicrobial therapy versus augmented immunosuppression).
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