[Acute on chronic respiratory failure in interstitial pneumonias].

BACKGROUND: Acute exacerbations of idiopathic interstitial pneumonias and exacerbations of pleuropulmonary disease in connective tissue diseases are associated with a high mortality. DIAGNOSTIC PROCEDURES: They have to be differentiated from respiratory failure in patients with interstitial lung disease as a result of infections, pulmonary embolism, cardiac failure and drug toxicity, because the latter can be treated causally. The extent and the invasivity of diagnostic procedures have to be adopted to the patient's situation. A rapid diagnosis is important in order to initiate therapies (antibiotics, anticoagulation, immunosuppression) in treatable causes of exacerbations. IMPORTANCE OF VENTILATION: The prognosis for patients who are under invasive mechanical ventilation in acute exacerbations of interstitial lung disease is poor. Especially in acute exacerbations of idiopathic pulmonary fibrosis (IPF), there is no general recommendation for either invasive or for noninvasive ventilation. In acute exacerbations of other subtypes of interstitial pneumonia, either idiopathic or as a pulmonary manifestation of connective tissue disease, and in all secondary exacerbations of idiopathic interstitial pneumonias which offer further treatment options, the decision about mechanical ventilation requires a differentiated assessment. LUNG TRANSPLANTATION: In younger patients with interstitial lung disease and a progressive disease, indication for lung transplantation should be made early and before an exacerbation. If patients listed for lung transplantation experience an acute exacerbation, bridging-to-transplant has to be discussed with the transplant unit. In cases without further causal treatment options palliative care must be initiated.