Issues of critical care involving the interstitial lung diseases (ILDs) are dominated by the progression of hypoxaemia and acute-on-chronic respiratory failure. Medical intensivists must often manage rapidly declining respiratory function caused by both the underlying interstitial process as well as superimposed complications, which are often related to co-morbidities. A commonly recognized phenomenon is acute exacerbation (AE), which is defined by the appearance of new diffuse pulmonary infiltrates superimposed on radiologic changes of underlying lung fibrosis accompanied by acute dyspnoea and worsening hypoxaemia that are not attributable to a secondary cause. An AE event is associated with high morbidity and mortality, particularly if mechanical ventilation is required. Co-morbidities such as pulmonary hypertension (PH) with related congestive heart failure (CHF), cardiac ischaemia, community-acquired infection, and deep-vein thrombosis/pulmonary embolism (DVT/PE) may also cause or contribute to clinical worsening, leading to intensive care unit (ICU) admission. This chapter highlights the incidence and presentation of AE and other known co-morbidities associated with ILD that may contribute to critical illness. This is followed by a review of current management strategies including the delineation of secondary causes of respiratory failure and a discussion of the evidence and rationale for supportive treatments such as antibiotics, high-dose intravenous (IV) steroids, and mechanical ventilation. Finally, a review of both in-hospital and long-term survival will be discussed along with end-of-life and palliative care perspectives.
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