Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients

Idiopathic pulmonary fibrosis (IPF) stands out as one of the most aggressive forms of interstitial lung diseases (ILDs), currently without a definitive cure. Multidisciplinary discussion (MDD) is now considered a cornerstone in diagnosing and differentiating ILD subtypes. The Gender-Age-Physiology (GAP) score, developed to assess IPF prognosis based on sex, age, forced vital capacity, and diffusion capacity for carbon monoxide (DLCO), is limited in not considering dyspnea and functional impairment during the walking test. We proposed a MDD-based clinical score for mortality prediction among those patients.

From December 2018 to December 2019, we enrolled ILD patients with IPF and non-IPF and followed-up them till December 2020. Based on DLCO, modified Medical Research Council (mMRC) Dyspnea Scale, and six-minute walking test (6MWT) distance, a functional score was developed for mortality prediction.

We enrolled 104 ILD patients, 12 (11.5%) died by the one-year follow-up. In receiver operating characteristic (ROC) curve analysis, DLCO (% predicted) was the most accurate variable predicting one-year mortality with an area under curve (AUC) of 0.88 (95% confidence interval [CI] = 0.80–0.94), followed by mMRC Dyspnea Score (AUC = 0.82 [95% CI = 0.73–0.89]), 6MWT distance (AUC = 0.80 [95% CI = 0.71–0.88]), and GAP score (AUC = 0.77 [95% CI = 0.67–0.84]). Only the GAP score (hazard ratio [HR] = 1.55, 95% CI = 1.03–2.34, p = 0.0.37) and functional score (HR = 3.45, 95% CI = 1.11–10.73, p = 0.032) were significantly associated with one-year mortality in multivariable analysis.

The clinical score composite of DLCO, mMRC Dyspnea Scale, and 6MWT distance could provide an accurate prediction for long-term mortality in ILD patients, laying out a helpful tool for managing and following these patients.