How should I manage a patient with interstitial lung disease who presents with acute exacerbation?

Management of a patient with interstitial lung disease (ILD) presenting with an acute exacerbation requires prompt clinical assessment and supportive care tailored to the severity of the exacerbation and underlying disease. First, assess the severity of respiratory symptoms, oxygen saturation, and signs of respiratory failure, using pulse oximetry and arterial blood gases if indicated, to guide oxygen therapy and the need for hospital admission. Oxygen should be administered cautiously to maintain target saturations, typically 88–92%, to avoid hypercapnic respiratory failure, especially if there is coexisting chronic lung disease ¹.

Hospital admission is generally indicated for severe breathlessness, hypoxia, or inability to cope at home. In the hospital setting, supportive care includes oxygen supplementation, careful monitoring, and consideration of ventilatory support; however, mechanical ventilation, including non-invasive ventilation, is usually not recommended routinely in ILD exacerbations due to poor outcomes and should be discussed sensitively with the patient and family ³.

Pharmacological treatment options for acute exacerbations of ILD are limited and not well established. Corticosteroids are often used empirically, although evidence is limited; their use should be balanced against potential risks. Management should also include treatment of any identifiable triggers such as infection or heart failure, and exclusion of other causes of acute deterioration [1, (Mathai and Danoff, 2016)].

Supportive measures such as pulmonary rehabilitation and symptom control should be considered once the acute phase is managed, and referral to specialist ILD services is important for ongoing care, including consideration of lung transplantation in appropriate candidates ³. Best supportive care, including palliative care involvement, should be integrated early, especially given the poor prognosis associated with acute exacerbations ³.