Δείκτες παρακολούθησης και πρόγνωσης στην ιδιοπαθή πνευμονική ίνωση

Abstract

Background: In Idiopathic Pulmonary Fibrosis (IPF) irreversibly progressive fibrosingparenchymal damage, leads to defects in mechanics and gas exchange, manifesting withdisabling exertional dyspnea. Previous studies have shown a relationship betweenfibroblast foci (FF) profusion and disease severity and survival. We hypothesized arelationship between Medical Research Council (MRC) dyspnea scale with FF, and arelationship between FF and functional parameters and survival.Methods: We retrospectively reviewed 24 histologically documented IPF patients.Profusion of FF was semiquantitatively evaluated by two scores, Brompton andMichigan. Survival analysis was performed by fitting Cox regression models toexamine the relationship of the two scores with survival and the non-parametricSpearman correlation coefficient was calculated to describe the relationships of FFscores with dyspnea scores and functional parameters.Results: No statistically significant correlation between FF scores and the MRC scoreswas observed (p = 0.96 and p = 0.508 respectively). No significant correlation betweenFF scores and survival (p = 0.438 and p = 0.861 respectively) or any functionalparameter was observed.Conclusions: The lack of relationship between the MRC dyspnea scale and the FFmight relate to the fact that dyspnea in IPF better reflects the overall of lung damageand its related consequences on mechanics and gas exchange whereas FF, one of itshistological hallmarks, may not reflect its entire histology derangement also constrainedby the geographically limited sampled tissue. This might be also valid for the observedlack of association between FF and survival or functional parameters.