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

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

Abstract

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.

Figures

Fig. 1
Fig. 1
Kaplan-Meier survival curves of patients with IPF acute exacerbation ever treated (black line), never treated (red line) with steroids and immunosuppressants and of the overall population (green line) (p = 0.022)
Fig. 2
Fig. 2
Kaplan-Meier survival curves of patients with IPF acute exacerbation treated with the study protocol after hospital discharge (black line) that showed an 83 % 1-year survival. Patients that died while hospitalized are shown in red line and the overall IPF acute exacerbation population in green line

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