title: Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach.
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.
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. papiris@otenet.gr.
authors.forename: Spyros A
authors.initials: SA
authors.lastname: Papiris
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. konstantinos.kagouridis@gmail.com.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Kagouridis
authors.affiliation: 7th Pulmonary Department and Asthma Center, Sotiria Chest Diseases Hospital, Athens, Greece. lykol@yahoo.gr.
authors.forename: Likurgos
authors.initials: L
authors.lastname: Kolilekas
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. papioannouandriana@gmail.com.
authors.forename: Andriana I
authors.initials: AI
authors.lastname: Papaioannou
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. anezaroussou@yahoo.com.
authors.forename: Aneza
authors.initials: A
authors.lastname: Roussou
authors.affiliation: 6th Pulmonary Department, Sotiria Chest Diseases Hospital, Athens, Greece. xristina.triantafyllidou@gmail.com.
authors.forename: Christina
authors.initials: C
authors.lastname: Triantafillidou
authors.affiliation: 4th Pulmonary Department, Sotiria Chest Diseases Hospital, Athens, Greece. katebaou@gmail.com.
authors.forename: Katerina
authors.initials: K
authors.lastname: Baou
authors.affiliation: Imaging and Research Unit, Evgenidion University Hospital, National and Kapodistrian University of Athens, Athens, Greece. kmalag@otenet.gr.
authors.forename: Katerina
authors.initials: K
authors.lastname: Malagari
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. steliosargentos@yahoo.gr.
authors.forename: Stylianos
authors.initials: S
authors.lastname: Argentos
authors.affiliation: 1st Department of Critical Care, Evangelismos Hospital, National and Kapodistrian University of Athens, Athens, Greece. akotanid@med.uoa.gr.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Kotanidou
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. annakara@otenet.gr.
authors.forename: Anna
authors.initials: A
authors.lastname: Karakatsani
authors.affiliation: 2nd Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece. fmanali@otenet.gr.
authors.forename: Effrosyni D
authors.initials: ED
authors.lastname: Manali
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000401:mortality*; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D013256:Steroids / Q000008:administration & dosage* / Q000009:adverse effects*; D015996:Survival Rate
chemical_list: D013256:Steroids