Health-related quality of life of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Idiopathic pulmonary fibrosis (IPF) is one of the most common forms of interstitial lungdisease presenting in persons 50 years and older. Through a comprehensive review of available studies, weaimed to assess health-related quality of life (HRQoL) of people living with IPF and the instruments usedin this assessment. Searches were conducted up to May, 2020. Quality appraisal and data extraction wereperformed using pre-designed forms. Narrative synthesis approach was used to report results of thesystematic review and a random effects model was used for the meta-analysis. A leave-one-out sensitivityanalysis was performed, and a trim and fill method was used to assess publication bias.The review included 134 studies. The most used instruments to measure HRQoL were St George’sRespiratory Questionnaire (SGRQ), Short Form 36 (SF36) and EuroQoL (EQ5D). Standardised meanscores (95% confidence interval) for these instruments were as follows: SGRQ total score: 44.72 (42.21–47.22); SF36 physical component score (PCS): 37.00 (34.74–39.26) SF36 mental component score (MCS):50.18 (48.41–51.95); King’s Brief Interstitial Lung Disease questionnaire total score: 58.38 (55.26–61.51);and EQ5D utility: 0.73 (0.68–0.79). Analysis of standardised means for both SGRQ and SF36demonstrated worse scores in physical health domains as compared to mental health domains.This systematic review confirms that IPF negatively affected HRQoL, mostly impacting the physicalhealth domains. This study also demonstrated that a diverse number of instruments are used to evaluateHRQoL. In view of this diversity, a standardised approach to measurement of HRQoL for IPF isimportant to ensure that comparisons made are reliable