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Introduction: Idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) are fibrotic ILDs with divergent disease populations. Little is known about health-related quality of life (HRQL) in SSc-ILD relative to IPF., Methods: We used the Kings Brief Interstitial Lung Disease Questionnaire (K-BILD) to compare HRQL in a cross-sectional study of 57 patients with IPF and 29 patients with SSc-ILD. Analysis of covariance was used to adjust for age, gender and lung function., Results: The unadjusted mean K-BILD score was 63.1 (95% CI 57.1 to 69.1) among patients with SSc-ILD, as compared with 54.7 (51.8-57.5) among those with IPF (p=0.005). However, this difference in HRQL was attenuated after adjustment for age, gender and lung function. In a multivariable model, only forced vital capacity was associated with K-BILD scores. K-BILD scores were correlated with both forced vital capacity and with other relevant HRQL measures, regardless of ILD diagnosis., Discussion: Patients with SSc-ILD may have better ILD-specific quality of life than patients with IPF, but this difference appears to be driven primarily by better lung function. These results underscore the impact of lung function on HRQL in fibrotic ILD and the utility of K-BILD to assess HRQL in SSc-ILD., Competing Interests: Competing interests: MTD reports research funding from Boehringer Ingelheim Norway KS, unrelated to the current study, lecture and consulting fees from Boehringer Ingelheim, Roche and AstraZeneca, unrelated to the current study, having participated in advisory boards for Boehringer Ingelheim and Roche, and that an immediate family member is a full-time employee of Boehringer Ingelheim who was not involved in this study. A-MH-V reports research funding and/or consulting fees or other remuneration from Actelion, Boehringer Ingelheim, Bayer, Roche, MSD and GlaxoSmithKline. TE reports receiving lecture fees from Boehringer Ingelheim and having participated in advisory boards for GlaxoSmithKline and Boehringer Ingelheim. A-OH and OEJ are full-time employees of Boehringer Ingelheim Norway KS. MBL and GB have no competing interests to report. SB reports research funding to his institution for the use of K-BILD in other studies, but not for the current study. TMJ reports receiving lecture fees from Boehringer Ingelheim and Roche. TS reports lecture fees from Boehringer Ingelheim and Roche., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by progressive loss of lung function. Its clinical course is variable but ultimately fatal. There is a need for a multicentre patient registry incorporating longitudinal clinical data and biological samples to improve understanding of the natural history of IPF and contemporary practice patterns., Methods/design: The Idiopathic Pulmonary Fibrosis-PRospective Outcomes (IPF-PRO) registry is a national IPF registry in the USA. This registry will enrol approximately 300 patients with newly diagnosed IPF over 2 years at approximately 14 tertiary pulmonary care sites. Participants will be followed for 3-5 years and will receive usual care, as defined by their physician. Clinical data from the year prior to diagnosis will be collected from medical record review on enrolment. Subsequently, data on diagnostic evaluations, pulmonary function tests, physical examinations, laboratory data and clinical events will be collected at routine clinical visits and via a call centre. Participants will complete patient-reported outcome questionnaires at enrolment and then at approximately 6-month intervals. Blood samples for cellular, genetic and transcriptomic analyses will be collected at the same intervals., Results: The first results from the IPF-PRO registry will be presented in 2015., Conclusions: The IPF-PRO registry will improve understanding of the natural history of IPF, its impact on patients and current practice in the diagnosis and care of patients with IPF. The registry will establish a repository of biological samples from a well-characterised patient population for future research., Clinical Trial Number: NCT01915511.