Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) carries a high risk of short-term mortality, and optimal management strategies remain unclear. Although recent studies have suggested that antifibrotic therapy improves outcomes following acute exacerbation, whether antifibrotic therapy continuation confers prognostic benefit in patients already receiving treatment at the time of AE remains unclear. In real-world practice, continuation during the acute phase is usually difficult due to adverse events and impaired oral intake.

This retrospective observational cohort study used the Japanese nationwide Diagnosis Procedure Combination database. It included patients hospitalized for AE-IPF between April 2018 and March 2023 and receiving nintedanib before admission. Patients who received nintedanib within 3 days of admission and continued treatment until day 7 were classified as the nintedanib-continued group and the remaining patients as the nintedanib-discontinued group. Propensity score matching was employed for baseline difference adjustment. The primary outcome was in-hospital mortality, whereas the secondary outcomes included survival time from admission to death or discharge and adverse events during hospitalization.

Of the 572 eligible patients, 181 continued and 391 discontinued nintedanib during hospitalization. After propensity score matching, 146 matched pairs were analyzed. In-hospital mortality did not significantly differ between the continued and discontinued groups. The median in-hospital survival time was identical in both groups (76 days). Adverse events were uncommon and did not significantly differ between the groups.

In this nationwide cohort of patients hospitalized for AE-IPF, nintedanib continuation during the acute phase did not result in improved short-term outcomes compared with discontinuation.