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Original article
Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease
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  1. Colas Tcherakian1,
  2. Vincent Cottin2,
  3. Pierre-Yves Brillet3,
  4. Olivia Freynet1,
  5. Nicolas Naggara3,
  6. Zohra Carton1,
  7. Jean-François Cordier2,
  8. Michel Brauner3,
  9. Dominique Valeyre1,
  10. Hilario Nunes1
  1. 1Université Paris 13, UPRES EA 2363, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France
  2. 2Hospices Civils de Lyon, Service de Pneumologie et Centre de Référence des Maladies Pulmonaires Rares, Hôpital Louis Pradel, Université Lyon 1, UMR 754, Lyon (Bron), France
  3. 3Université Paris 13, UPRES EA 2363, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Radiologie, Bobigny, France
  1. Correspondence to Hilario Nunes, Service de Pneumologie, Hôpital Avicenne, 125 rue de Stalingrad, 93009 Bobigny, France; hilario.nunes{at}avc.aphp.fr

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.

Conclusion 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.

  • Idiopathic pulmonary fibrosis
  • asymmetry
  • natural history
  • acute exacerbation
  • gastro-oesophageal reflux
  • interstitial fibrosis

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Footnotes

  • See Editorial, p 183

  • Linked articles 149096.

  • Competing interests DV has received consultancy fees from Intermune, Actelion and Sanofi (less than $4000 in the last 5 years) and expenses from AstraZenica, Glaxo, Boehringer and Nycomed for travel and accommodation at ATS, ERS and CPLF meetings.

  • Ethics approval This study was conducted with the approval of the institutional review board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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