A comparison of heath-related quality of life in IPF and systemic sclerosis-related ILD

Abstract

Introduction: IPF and systemic sclerosis (SSc)-related interstitial lung disease (ILD) are fibrotic ILDs with a poor prognosis. Assessment of health-related quality of life (HRQL) is important to understand disease trajectory and response to therapy in ILD; however, few studies have compared HRQL in these conditions. Objective: To compare HRQL in SSc-ILD and IPF, independent of lung function. Methods: The Kings Brief Interstitial Lung Disease Questionnaire (K-BILD), validated to assess HRQL in IPF and scored 0-100 with higher scores indicating better HRQL, was used to compare HRQL between patients with IPF (n=58) and SSc-ILD (n=29) in a multi-center cross-sectional study. ANCOVA was used to compare mean K-BILD score between the IPF and SSc-ILD groups, with adjustment for age, gender, FVC and DLCO. Values are presented as mean (SD). Results: Demographics and lung function are listed in table 1. K-BILD score was 69.4 (17.9) among patients with IPF and 80.5 (21.2) among those with SSc-ILD (p =0.014), indicating slightly better HRQL in SSc-ILD. After covariate adjustment, however, no statistical difference in K-BILD was observed between patients with IPF (70.5 [19.3]) and SSc-ILD (79.8 [21.2], p = 0.096). Conclusions: K-BILD scores appeared to be similar between patients with IPF and SSc-ILD after adjusting for age, gender and lung function. This new observation underscores the impact of lung function impairment on HRQL in ILD and the need for treatments that positively impact HRQL by preserving lung function.