Acute exacerbations of idiopathic pulmonary fibrosis

Acute exacerbations of idiopathic pulmonary fibrosis are defined as periods of acute clinically significant respiratory deterioration, accompanied by new radiological abnormalities on HRCT (ie bilateral ground-glass opacification or consolidation) on a background of a UIP pattern.

The annual incidence of acute exacerbations in patients with idiopathic pulmonary fibrosis may be up to 15%, although estimates vary. Risk factors include advanced disease, younger age and a history of previous acute exacerbations. Patients presenting with an acute exacerbation of idiopathic pulmonary fibrosis and associated respiratory failure have high mortality (greater than 50%).

The cause of acute exacerbations is unknown; potential triggers include infection, aspiration, thoracic surgical procedures, mechanical ventilation and drugs. Exclusion of respiratory infection and cardiac failure is important.

There are no proven effective therapies for acute exacerbations. Supportive measures include oxygen for hypoxaemia and management of dyspnoea. Although evidence to support corticosteroid therapy is lacking, it is often trialled given lack of alternative treatment options.