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.
Highlights
Idiopathic pulmonary fibrosis acute exacerbation (IPF-AE) constitutes IPF’s most devastating event, representing the unexpected superimposition of diffuse alveolar damage of unknown etiology
The protocol that we applied in every patient admitted with the diagnosis of IPF deterioration has been described in previous publications [1, 6] and consisted of immediate cessation of immunosuppression, best supportive care, broad spectrum antimicrobials according to the immune status of each patient, and thorough evaluation to detect reversible causes of respiratory deterioration
Patients never treated with steroids or/and immunosuppressants before the acute exacerbation event were set on antimicrobials for common gram positive, gram negative and atypical pathogens plus therapy against influenza virus pneumonia during the influenza season; patients previously treated with steroids or/and immunosuppressants received in addition to the above mentioned therapy, treatment for Pneumocystis jirovecii and occasionally for cytomegalovirus pneumonia in a documented case
Summary
Idiopathic pulmonary fibrosis acute exacerbation (IPF-AE) constitutes IPF’s most devastating event, representing the unexpected superimposition of diffuse alveolar damage of unknown etiology. We hypothesized that previous immunosuppression and the administration of high-dose steroids adversely affect IPF-AE outcome. IPF-AE histological picture corresponds to the development of diffuse alveolar damage (DAD) upon usual interstitial pneumonia (UIP) [2] This corresponds clinically to the development of an unknown etiology acute respiratory distress syndrome (ARDS) in IPF. Years before the PANTHER trial [5], the triple therapy strategy was abandoned in our everyday clinical practice and was appropriately supported in previous publications together with our adversity for the high-dose steroids regimen upon IPF-AE [1, 6]. All patients were followed-up for survival; after discharge none received immunosuppression
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