Journal Article

Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach

Papiris, Spyros A, Triantafillidou, Christina, Malagari, Katerina, Roussou, Aneza, Baou, Katerina, Kagouridis, Konstantinos, Argentos, Stylianos, Kotanidou, Anastasia, Manali, Effrosyni D, Papaioannou, Andriana I, Karakatsani, Anna, Kolilekas, Likurgos

2015

Overview

Background Idiopathic pulmonary fibrosis acute exacerbation (IPF-AE) constitutes IPF’s most devastating event, representing the unexpected superimposition of diffuse alveolar damage of unknown etiology. Guidelines recommend high-dose steroids treatment despite unproven benefit. We hypothesized that previous immunosuppression and the administration of high-dose steroids adversely affect IPF-AE outcome. Methods We studied all consecutive patients hospitalized in our department for IPF deterioration from 2007 to June 2013. Our protocol consisted of immediate cessation of immunosuppression (if any), best supportive care, broad-spectrum antimicrobials and thorough evaluation to detect reversible causes of deterioration. Patients were followed-up for survival; post-discharge none received immunosuppression. Results Twenty-four out of 85 admissions (28 %) fulfilled IPF-AE criteria. IPF-AE were analyzed both as unique events and as unique patients. As unique events 50 % survived; 3 out of 12 (25 %) in the group previously treated with immunosuppression whereas nine out of 12 (75 %) in the group not receiving immunosuppression ( p  = 0.041). As unique patients 35.3 % survived; 3 out of 6 (50 %) in the never treated group whereas three out of 11 (27.3 %) in the group receiving immunosuppression ( p  = 0.685). The history of immunosuppression significantly and adversely influenced survival ( p  = 0.035). Survival was greater in the never treated group compared to the immunosuppressed patients ( p  = 0.022). Post-discharge, our IPF-AE survivors had an 83 % 1-year survival. Conclusions By applying the above mentioned protocol half of our patients survived. The history of immunosuppression before IPF-AE adversely influences survival. Avoiding steroids in IPF patients may favor the natural history of the disease even at the moment of its most devastating event.

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V
• Subject: Aged / Aged, 80 and over / Analysis / Anti-infective agents / Critical Care Medicine / Development and progression / Disease Progression / Female / Humans / Idiopathic Pulmonary Fibrosis - drug therapy / Idiopathic Pulmonary Fibrosis - mortality / Immunosuppression - adverse effects / Immunotherapy / Infectious / Intensive / Internal Medicine / Kaplan-Meier Estimate / Male / Medical research / Medicine / Medicine & Public Health / Medicine, Experimental / Middle Aged / Pneumology/Respiratory System / Pulmonary fibrosis / Pulmonology / Rare and Idiopathic Pulmonary Diseases / Regression Analysis / Research Article / Steroids - administration & dosage / Steroids - adverse effects / Survival Rate