Outcome differences between idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD) - data from the EXCITING registry

Abstract

Background: Available epidemiological data suggest that IPF is a frequent and unfavourable fibrosing ILD. Yet, data on outcome differences between IPF and other ILDs are scant. Method: The prospective, multi-centre German ILD registry “EXCITING” was analysed for differences in outcomes between IPF and other ILDs. Results: Out of 601 patients (60% male, 53% ex-/smokers, medians: age 64 years, FVC 75%, DLCO 53%), 151 (25%) suffered from IPF (76% male, 58% ex-/smokers, medians: age 74 years, FVC 69%, DLCO 39%). Of 314 all-cause hospitalisations 32% occurred in IPF. Also, IPF accounted for 29% and 35% of respiratory and acute exacerbation (AE) related hospitalisations, respectively. Out of 91 deceased patients, 46% had IPF. Time to death was 101 months for IPF compared to 229 months for non-IPF ILDs (non-IPF, p 10%, hospitalization due to respiratory reason or death), differed between IPF vs. non-IPF with a mean of 62 months vs. 113 months (p=0.006). In IPF, there was also a trend for differences in PFS related to baseline FVC: for FVC 90% 107 months (p=0.077). Conclusions: IPF is a common ILD subtype with frequent hospitalisations, more rapid progression and worse mortality than other ILDs.