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Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease.
MedLine Citation:
PMID:  20880868     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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&emsp14;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.
Authors:
Colas Tcherakian; Vincent Cottin; Pierre-Yves Brillet; Olivia Freynet; Nicolas Naggara; Zohra Carton; Jean-François Cordier; Michel Brauner; Dominique Valeyre; Hilario Nunes
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Publication Detail:
Type:  Journal Article     Date:  2010-09-29
Journal Detail:
Title:  Thorax     Volume:  66     ISSN:  1468-3296     ISO Abbreviation:  Thorax     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-02-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417353     Medline TA:  Thorax     Country:  England    
Other Details:
Languages:  eng     Pagination:  226-31     Citation Subset:  IM    
Affiliation:
Service de Pneumologie, Hôpital Avicenne, 125 rue de Stalingrad, 93009 Bobigny, France. hilario.nunes@avc.aphp.fr.
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