Methylprednisolone pulse therapy in Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a fibrotic interstitial lung disease (ILD) with unknown pathogenesis and poor prognosis. Unpredictable events defined as Acute Exacerbations (AE-IPF) may be experienced up to one in five IPF patients. The hallmark is a rapid respiratory failure not fully explained by heart failure/infections and associated with new lung infiltrates. Guidelines recommended treatment with steroids despite a lack of solid evidence. Aims: To compare survival of AE-IPF patients treated with methylprednisolone to those not treated with steroids. Methods: Retrospective analysis of IPF patients admitted to the pulmonary ward from 1 Jan 2014 through 15 Jan 2020. A case-by-case record review was performed. Survival was analyzed using Cox regression and Kaplan-Meier. Results: We admitted 119 ILD patients due and 73 (61.3%) had IPF. We analyzed 26 (35.6%) AE-IPF subjects. Males were predominant (80.1%) with a mean age of 73.2 years and mostly smokers (63.2%). We administered steroid in 12 subjects (46.2%). 10 AE-IPFs died during hospitalization. No association between steroid and mortality was identified either during in-hospital stay (HR 0.81; CI 0.21-3.10; p=0.76). Conclusions: We found no association between corticosteroid use and improved survival in subjects with AE-IPF. Large, controlled-studies are needed to address this unsolved problem. To date, the decision to use steroids remains difficult