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

Abstract

BackgroundIdiopathic 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.MethodsWe 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.ResultsTwenty-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.ConclusionsBy 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.

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

“…DONAHOE et al [101] performed a pilot study to evaluate the efficacy of plasma exchange, rituximab and intravenous immunoglobulin for AE-IPF, and showed better one-year survival than historical controls without serious adverse events. On the other hand, based on the results of the PANTHER trial, which demonstrated that the combination of prednisone, azathioprine and N-acetyl cysteine was harmful for IPF [102], PAPIRIS et al [103] hypothesised that previous immunosuppression and the administration of high-dose steroids adversely affect outcome of AE-IPF, and studied a protocol of immediate cessation of immunosuppression (if any), best supportive care and broad-spectrum antimicrobials. Their uncontrolled results revealed that the steroid avoidance strategy might be of benefit.…”

Corticosteroids in acute exacerbations of idiopathic interstitial pneumonias: Time to debate – Reply

“…4 In keeping with this, a recent observational study of AE-IPF reported that patients never treated with immunosuppressants and steroids before and after an AE-IPF present a higher survival rate than those treated with steroids. 5 Contrary to this, in their study, Arai et al…”

“…5 However, in their study, three patients with AE-IPF and multiple events were included as 10 separate patients of AE-IPF. Furthermore, there was no significant difference in survival from the onset of the first event of AE between AE-IPF patients who had not (n = 6) and had (n = 11) been treated with corticosteroids (P = 0.640).…”

“…Furthermore, there was no significant difference in survival from the onset of the first event of AE between AE-IPF patients who had not (n = 6) and had (n = 11) been treated with corticosteroids (P = 0.640). 5 Our recommendation to treat AE-IIP with high-dose prednisolone is provisional because our study was retrospective with a limited number of patients. Our results should be confirmed in prospective randomized controlled trials.…”

Idiopathic pulmonary fibrosis acute exacerbations: where are we now?

“…Knowing how powerful properly conducted studies financed by scientific sources such as the National Heart, Lung and Blood Institute are to provide medical knowledge and to reduce bias, we strongly believe that in IPF, a study similar to the CORTICUS and PANTHER studies, comparing the two arms of the steroid and nonsteroid approach in the therapeutic management of 'acute exacerbation' of IPF is greatly awaited by scientists and owed to the patients. Uncontrolled preliminary clinical data appear to shed doubts upon the steroids approach on IPF acute exacerbations [24] In conclusion, the scientific community needs evidence whether IPF 'acute exacerbations' represent an intrinsic process of the disease or not because this will have enormous diagnostic and most importantly therapeutic implications. Certainly, by changing approach, we are not going to save IPF patients but according to Hippocrates 'do not harm' them.…”