Introduction: IPF is associated with impairment in HRQL.

Aim: To evaluate trajectories of HRQL in a real-world cohort of patients with IPF.

Methods: Patients with IPF that was diagnosed or confirmed at the enrolling centre in the previous 6 months were enrolled into the prospective IPF-PRO Registry at 46 sites across the USA. HRQL was assessed using the St George?s Respiratory Questionnaire (SGRQ) total score. The mean trajectory of SGRQ total score was estimated using a mixed joint model.

Results: The analysis cohort comprised 980 patients. Median (Q1, Q3) SGRQ total score at enrolment was 39.6 (25.5, 53.7). There was a non-linear relationship between SGRQ total score and time. Based on a non-linear model, the estimated mean change in SGRQ total score over 48 months was 13.6. Younger age, female sex, lower FVC % predicted, and particularly oxygen use were associated with a worse (higher) SGRQ total score at enrolment, but not with different trajectories of scores over time. The estimated difference in SGRQ total score at enrolment between patients who were and were not using oxygen at rest was 21.2; for oxygen use with activity, the difference was 13.1.

Conclusions: Prospective data from the IPF-PRO Registry suggest that among patients with IPF, younger age, female sex, lower FVC and oxygen use were associated with worse HRQL at enrolment, but not with different trajectories of HRQL over time.