title: Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease.
abstract: BACKGROUND
  In idiopathic pulmonary fibrosis (IPF) the distribution and spatial-temporal progression of fibrotic changes may be influenced by general or locoregional conditions. From this perspective, patients with asymmetrical disease (AIPF) may be unique.
  
  
  METHODS
  This retrospective study included 32 patients (26 men, mean ± SD age 69 ± 7 years) with AIPF, as defined by an asymmetry ratio (most affected--least affected fibrosis score)/(most affected + least affected fibrosis score) >0.2. The global fibrosis score was the average of the right and left scores. Patients with AIPF were compared with 64 matched controls with symmetrical IPF.
  
  
  RESULTS
  Patients with AIPF did not differ from controls in global fibrosis score and forced vital capacity, but carbon monoxide transfer factor was less decreased (52 ± 19% vs 43 ± 13%, p=0.009). The rate of gastro-oesophageal reflux and acute exacerbations was significantly higher in patients with AIPF (62.5% vs 31.3%, p=0.006 and 46.9% vs 17.2%, p=0.004, respectively). In patients with AIPF the right side was more likely to be involved (62.5%); the median asymmetry ratio was 0.5 (range 0.24-1). Although the global fibrosis score worsened significantly in all 23 patients with AIPF with serial high-resolution CT scans (p<0.0001), pulmonary fibrosis remained asymmetrical in all except three. During follow-up, 15 patients with AIPF experienced 18 acute exacerbations. The first episode was virtually unilateral, occurring in the most affected lung in 10 patients (66.7%). Survival was similar between patients with AIPF and controls.
  
  
  CONCLUSIONS
  AIPF may be related to locoregional factors including gastro-oesophageal reflux which may be responsible for both disease expansion and the occurrence of acute exacerbations.
authors.affiliation: Service de Pneumologie, Université Paris 13, UPRES EA 2363, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Bobigny, France.
authors.forename: Colas
authors.initials: C
authors.lastname: Tcherakian
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Brillet
authors.forename: Olivia
authors.initials: O
authors.lastname: Freynet
authors.forename: Nicolas
authors.initials: N
authors.lastname: Naggara
authors.forename: Zohra
authors.initials: Z
authors.lastname: Carton
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.forename: Michel
authors.initials: M
authors.lastname: Brauner
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
authors.forename: Hilario
authors.initials: H
authors.lastname: Nunes
mesh_terms: D000208:Acute Disease; D000368:Aged; D004812:Epidemiologic Methods; D005260:Female; D005602:France / Q000453:epidemiology; D005764:Gastroesophageal Reflux / Q000150:complications; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D014057:Tomography, X-Ray Computed