RT Journal Article
SR Electronic
T1 BAL markers of alveolar/capillary abnormality in IPF
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P3622
VO 40
IS Suppl 56
A1 Ermanno Puxeddu
A1 Alessia Comandini
A1 Paola Rogliani
A1 Assunta Porretta
A1 Francesco Cavalli
A1 Cesare Saltini
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P3622.abstract
AB Several lines of evidence suggest that alveolar-capillary abnormalities, including increased alveolar septal capillary density and pulmonary veno-occlusive disease,are characterizing feature of IPF and may play a role in its progression.This study assess altered capillary permeability,abnormal intra-alveolar coagulation and alveolar hemorrhage as markers of alveolar/capillary abnormality.Methods: Bronchoalveolar lavage (BAL) samples from 62 subjects (53 IPF patients and 14 healthy volunteers) were evaluated for α2-macroglobulin (α2-M) and fibrinogen D-dimer (D-d) concentration by ELISA.D-d levels were comparatively assessed in blood as well.The numbers of haemosiderin laden macrophages were measured by Perls' stain and the intensity thereof assessed by the Golde score.Results: IPF patients had markedly increased α2-M levels (mean 10000 vs 50 ng/ml, p<0,0001) and D-d were elevated with significantly higher frequency (39/62 vs 1/14, p<0,05) with no blood elevation.Golde scores were elevated (69 vs 19, p<0,001) compared to controls. α2-M concentration positively correlates with the Golde score (p<0,05) and D-d concentration (p<0,05).In patients with a high Golde score (Golde score>59) the D-d concentration (125 vs 17 p<0,05) was increased and both DLCO (43 vs 56%,p<0,05) and exercise capability (6MWTD 273 meters vs 415,p<0,05) were reduced vs patients with low score,while the FVC was not significantly different (82 vs 66%).Golde score and arterial pulmonary pressure showed significant correlation (R=0,39).Conclusions: leak of α2-M,intra-alveolar hemorrage and coagulation,indicate that alveolar-capillary abnormalities are important in the pathogenesis and progression of pulmonary fibrosis, and likely pulmonary hypertension in IPF.