Outcomes of patients with acute exacerbations of idiopathic pulmonary fibrosis treated with corticosteroids: a systematic review and meta-analysis

Background: Acute exacerbation of idiopathic pulmonary fibrosis (IPF) is the most severe complication and one of the main causes of death in these patients. No proven effective therapies have been reported until now but high-dose corticosteroids are suggested by the international guidelines. Methods: A search of the scientific evidence was carried out using PubMed, Embase, and Scopus. Observational and experimental studies describing clinical outcomes in adult patients with acute exacerbations of IPF treated with corticosteroids were included. Results: 32 studies were selected for the qualitative and quantitative analysis. In patients treated only with corticosteroids, the pooled 90-day and in-hospital mortality rate was 42% (95% CI = 19-67%) and 43% (95% CI = 30-56%), respectively. Pooled 90-day mortality in patients treated with methylprednisolone at doses of 1000 mg/day was higher than in those treated with 500-1000 mg/day (i.e. 10 mg/kg/day) (54% (95% CI = 11-94%) VS. 39% (95% CI = 12 to 71%)). In patients with background antifibrotic therapy, the pooled 90-days mortality was of 39% (95% CI = 25-54%) while in those without was of 49% (95% CI = 10-89%). The 90-day and in -hospital mortality for patients receiving concomitant immunosuppressive therapy was 37% (95% CI = 29-46%) and 35% (95% CI = 12-62%), respectively. The overall 1-year mortality was 43% (95%CI = 30-56%). Conclusions: Patients with acute exacerbation of IPF treated with corticosteroids show a high short- and long-term mortality. Those treated with lower steroid doses and with background antifibrotic therapy show the highest short-term survival rates.