RT Journal Article
SR Electronic
T1 Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease
JF Thorax
JO Thorax
FD BMJ Publishing Group Ltd and British Thoracic Society
SP 226
OP 231
DO 10.1136/thx.2010.137190
VO 66
IS 3
A1 Tcherakian, Colas
A1 Cottin, Vincent
A1 Brillet, Pierre-Yves
A1 Freynet, Olivia
A1 Naggara, Nicolas
A1 Carton, Zohra
A1 Cordier, Jean-François
A1 Brauner, Michel
A1 Valeyre, Dominique
A1 Nunes, Hilario
YR 2011
UL http://thorax.bmj.com/content/66/3/226.abstract
AB 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.