Respiratory Conditions: Interstitial Lung Disease

Interstitial lung disease (ILD) includes approximately 100 separate conditions that fall into four main categories: conditions with known etiologies (eg, connective tissue disease), granulomatous diseases, idiopathic interstitial pneumonias, and miscellaneous conditions. Most patients report unexplained exertional dyspnea that develops insidiously over a variable period. Cough also is common. Because the clinical manifestations of ILD mimic those of other lung diseases, comprehensive testing almost always is required. Testing typically includes chest imaging, pulmonary function testing, and basic laboratory tests. If findings are not consistent with common diagnoses, such as chronic obstructive pulmonary disease, additional testing with high-resolution computed tomography scan and bronchoscopy or surgical lung biopsy can help confirm the diagnosis and type of ILD. Depending on the type, therapy can involve management of the underlying disease (eg, management of an autoimmune condition) or symptomatic treatment. Several drugs and interventions are available to help alleviate symptoms, slow progression, and, in some cases, reverse the condition. In cases of refractory disease, lung transplantation may be required. For patients with progressive disease and contraindications to transplantation, palliative care measures should be considered.