AE of IPF (AE-IPF) represents episodes of acute and rapid respiratory deterioration without identifiable aetiology during the clinical course of IPF, and is burdened by a high mortality rate. It represents the leading cause of hospitalisation and death among patients with IPF. Currently available therapeutic strategies are poorly effective, and guidelines recommend best supportive care in association with high-dose steroid therapy, although the strength of the latter recommendation is weak. We review the available evidence on the treatment of AE-IPF, emphasising the therapeutic options supported by the current guidelines and describing new therapeutic approaches provided by various emerging drugs. We also examine the main points related to AE-IPF supportive care. Finally, we review the current approaches to prevention of AE-IPF.

Cite as: Condoluci C, Inchingolo R, Mastrobattista A, et al. IPF: treatment and prevention of pulmonary exacerbations. In: Burgel P-R, Contoli M, López-Campos JL eds. Acute Exacerbations of Pulmonary Diseases (ERS Monograph). Sheffield, European Respiratory Society, 2017; pp. 199–223 [https://doi.org/10.1183/2312508X.10002017].