Purpose of review: Respiratory failure associated with interstitial lung disease (ILD) occurs commonly, often as a terminal event after a prolonged course of illness. Diagnosis and management of the underlying ILD and respiratory failure pose great challenges.
Recent findings: Respiratory failure in the absence of a clearly identifiable cause has a high mortality and frequent complications. Patients with idiopathic pulmonary fibrosis who are admitted with respiratory failure have a grim prognosis and may not benefit from prolonged aggressive therapy including mechanical ventilation. Presence of diffuse alveolar damage or usual interstitial pneumonia on lung biopsy specimens from patients with respiratory failure may be a marker of poor prognosis. Recently, the importance of the clinical-radiologic-pathologic diagnosis has been emphasized.
Summary: The prognosis and treatment may vary according to the type of ILD and the cause of the respiratory failure, which must therefore be established before treatment is initiated. Prevention of iatrogenesis and timely application of palliation are as important as specific treatment of underlying ILD.