K-BILD is Associated With Mortality Risk in Patients With Fibrotic Interstitial Lung Disease Receiving Antifibrotic Therapy: A Multi-center Prospective Longitudinal Study
Abstract Background: Patients with fibrotic interstitial lung diseases (ILDs) often experience impaired health-related quality of life (HRQoL). The King's Brief Interstitial Lung Disease Questionnaire (K-BILD) has been developed to measure disease-specific HRQoL. However, its clinical application in real-world settings has not been fully explored. Methods: This prospective longitudinal study enrolled 290 patients with fibrotic ILD who initiated antifibrotic therapy. HRQoL was assessed using K-BILD at the start of antifibrotic therapy, at 6 months, and at 1 year. Associations between K-BILD scores, pulmonary function, and mortality risk were evaluated. Results: K-BILD was assessed in 284, 159, and 154 patients at the start of antifibrotic therapy, at 6 months, and at 1 year, respectively. The median K-BILD scores were 63, 67, and 67 points at the respective time points. At 6 months and 1 year, there were increases of 10 points or more in K-BILD in 45 and 41 patients, respectively. Conversely, there were decreases of 10 points or more in 30 and 42 patients, respectively. K-BILD was moderately associated with pulmonary function tests at the start of antifibrotic therapy; however, only weak correlations were found between changes in K-BILD and %FVC over time. Patients with K-BILD scores in the lowest tertile had significantly shorter survival compared to those in higher tertiles (median survival: 27.8 vs. 40.6 months). A longitudinal decrease in K-BILD of ≥10 points was associated with shorter survival. Multivariate analyses indicated that K-BILD was associated with mortality risk independent of ILD-GAP stage at the start of antifibrotic therapy. Conclusion: Antifibrotic therapy improves HRQoL in certain patients with fibrotic ILDs. Assessing K-BILD may be useful for monitoring mortality risk independently of pulmonary function tests.
Read more