Acute exacerbations complicating interstitial lung disease

Purpose of review The purpose of this review is to provide an update on acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), with a specific focus on new data regarding the cause, clinical features, management and prognosis of AE-IPF. In addition, the limitations of the current definition of AE-IPF are discussed and a novel classification schema is proposed. Recent findings AE-IPF occurs in up to 15% of IPF patients annually and has a mortality of approximately 50%. The incidence of AE-IPF is higher in patients with worse lung function and may be increased in some populations. Emerging data suggest that exacerbations may be secondary to subclinical triggers such as infection, aspiration, mechanical injury and air pollution. Management of AE-IPF typically includes high-dose corticosteroids and antimicrobials; however, there are limited data to support these or other therapies. Prevention of AE-IPF with antifibrotic medications may be feasible and warrants further study. Summary AE-IPF is associated with significant morbidity and mortality; however, there remains a paucity of clinical data. The current definition of AE-IPF has limitations and a new classification schema should be considered.