title: Acute exacerbation of idiopathic pulmonary fibrosis: outcome and prognostic factors.
abstract: BACKGROUND
  Acute exacerbation is a substantial cause of death in patients with idiopathic pulmonary fibrosis with poorly described prognostic factors.
  
  
  OBJECTIVE
  To review the features associated with acute exacerbation of idiopathic pulmonary fibrosis and assess its prognostic factors.
  
  
  METHODS
  Thirty-seven occurrences of acute exacerbation of idiopathic pulmonary fibrosis were retrospectively reviewed in the medical records of 27 patients. Clinical presentation, radiographic studies, pulmonary function tests, laboratory data, treatment, and outcome were analyzed.
  
  
  RESULTS
  Acute exacerbation of idiopathic pulmonary fibrosis occurred more frequently between December and May (75.7%) than between June and November (24.3%) (p = 0.01). In-hospital mortality was 27% and median survival was 4.2 months (range 0.2-36.6). Significant differences between nonsurvivors and survivors included the time elapsed between their admission and the initiation of treatment for acute exacerbation (6 vs. 3.1 days, p = 0.04), lactate dehydrogenase levels at admission (801 vs. 544.6 IU/l, p = 0.002), impairment of the prior forced vital capacity (51.2 vs. 65%, p = 0.01) and diffusing capacity for carbon monoxide (21.7 vs. 34%, p = 0.01). Furthermore, the evolution of gas exchange in the first 10 days after the initiation of treatment was associated with in-hospital and long-term mortality.
  
  
  CONCLUSIONS
  Acute exacerbations of idiopathic pulmonary fibrosis are more frequent during winter and spring. The time between admission and initiation of treatment is a new reported prognostic factor that should be investigated further. This finding highlights the need for a fast diagnostic approach that should probably be standardized. Early gas exchange modifications reflect the response to treatment and predict the prognosis.
authors.affiliation: Service de Pneumologie, Hôpital Universitaire Avicenne, Assistance Publique-Hôpitaux de Paris, Bobigny, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Simon-Blancal
authors.forename: Olivia
authors.initials: O
authors.lastname: Freynet
authors.forename: Hilario
authors.initials: H
authors.lastname: Nunes
authors.forename: Diane
authors.initials: D
authors.lastname: Bouvry
authors.forename: Nicolas
authors.initials: N
authors.lastname: Naggara
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Brillet
authors.forename: Damien
authors.initials: D
authors.lastname: Denis
authors.forename: Yves
authors.initials: Y
authors.lastname: Cohen
authors.forename: François
authors.initials: F
authors.lastname: Vincent
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Naccache
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D002648:Child; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D017052:Hospital Mortality / Q000639:trends; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012008:Recurrence; D012129:Respiratory Function Tests; D012138:Respiratory Therapy / Q000379:methods*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D007166:Immunosuppressive Agents