Determinants of the prognosis of idiopathic pulmonary fibrosis

OBJECTIVE: Fibrotic idiopathic interstitial pneumonias are chronic and pro - gressive lung diseases with different progno - sis, with idiopathic pulmonary fibrosis (IPF) having the worst prognosis. Many patients need a surgical lung biopsy for the definite di- agnosis of IPF but age and the clinical context often contraindicate this procedure.The aim of this study is to identify predictors of survival, apartfromlungbiopsy,inpatientswithdefinite andpossibleIPF. PATIENTS AND METHODS :Westudied42pa - tientswithHRCTpatternofdefiniteorpossible IPF, by assessing the mortality in relationship with baseline HRCT and functional findings. HRCT was assessed both as prevalent pattern (definite vs possible UIP) and as score of the different abnormalities (in particular, honey - combing (HC) and total fibrotic score). Pul- monary function was assessed as baseline FVC,TLC and DLCO values, as well as change over6monthsoffollow-up.Bothunivariateand multivariate analyses were performed in order todetectpredictorsofmortality. RESULTS :Duringfollow-up,10outof42patients died.Mortalityratewasnotdifferentaccordingto thequalitativepatternoffibrosisatHRCT.Among thedifferentHRCTscores,acut-offof15%inthe HC score differentiated patients with higher mor- talityrate.AlowerbaselineFVC,andagreaterde - creaseinpulmonaryfunctionafter6months,were bothassociatedwithhighermortality.Inalogistic analysistakinginconsiderationclinical,radiologi- calandfunctionalfindings,onlybaselineFVCand FVC change after 6 months resulted significant predictorsofmortality. CONCLUSIONS: Functional evaluation at the baseline and during follow-up is more relevant than HC score for the prognosis of patients withdefiniteandpossibleIPF.