Abstract Background Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a life-threatening event with poor prognosis. Several prognostic factors at the onset of AE have been reported; however, prognostic determinants among patients who survive an AE episode remain unclear. Methods We retrospectively reviewed 102 patients with first-episode AE-IPF who required hospitalization at our institution between 2010 and 2024. Of these, 33 patients died during hospitalization and 69 were discharged alive. Among the survivors, 30 patients who were alive at 6 months after AE onset and had both pulmonary function tests (within ±3 months) and dyspnea assessments available at that time point were included for analysis. Clinical variables, including age, sex, forced vital capacity (FVC), percent predicted FVC (%FVC), oral prednisolone dose, and modified Medical Research Council (mMRC) dyspnea scale, were evaluated for association with post-AE survival using Cox proportional hazards models. Results The cohort included 22 men (84.6%) with a mean age of 73.5 ± 7.8 years, FVC = 2.45 ± 0.62 L, and %FVC = 76.9 ± 16.5%. The median post-AE survival time was 42.7 ± 31.1 months. Higher dyspnea scale (mMRC) at 6 months after AE was significantly associated with shorter subsequent survival (HR 1.78, 95% CI 1.06-2.96, p = 0.03). Age (HR 1.02, 95% CI 0.97-1.08, p = 0.48), male sex (HR 2.41, 95% CI 0.70-8.33, p = 0.13), %FVC (HR 0.99, 95% CI 0.97-1.02, p = 0.59), and daily prednisolone dose (HR 1.01, 95% CI 0.95-1.09, p = 0.77) were not significantly associated with survival. Importantly, mMRC remained a significant predictor even after adjustment for age, sex, and %FVC, respectively. Conclusion Among survivors of AE-IPF, mMRC scale at 6 months post-AE is a strong independent predictor of long-term prognosis. These findings highlight the potential role of persistent dyspnea as a clinical marker reflecting residual pathophysiological impairment following AE, warranting further investigation into its underlying mechanisms. This abstract is funded by: Fukuda Denshi Co.,Ltd.
Nishiyama et al. (Fri,) studied this question.