Nintedanib for progressive pulmonary fibrosis associated with sarcoidosis: a retrospective preliminary observational report.

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology. The efficacy of antifibrotic drugs for progressive pulmonary fibrosis (PPF) associated with sarcoidosis has not yet been sufficiently clarified. Therefore, we retrospectively examined the effectiveness and safety of nintedanib in patients with pulmonary sarcoidosis who met PPF criteria. METHODS: Participants comprised six patients with sarcoidosis complicated by pulmonary fibrosis who were treated with nintedanib for PPF between 2020 and 2023. The forced vital capacity (FVC) change rate (mL/year) before and after nintedanib initiation was compared using the Wilcoxon signed-rank test. RESULTS: Among the six patients (5 male; median age at nintedanib initiation, 59 years), two had autoimmune pulmonary alveolar proteinosis. Oral prednisolone (2-7.5 mg daily) was simultaneously administered in five patients. The initial nintedanib doses were 200 mg (n = 3) and 300 mg daily (n = 3). The median annual declines in FVC before and after nintedanib initiation were -254.0 mL/year and -65.9 mL/year, respectively. Although this difference did not reach significance, the FVC increased in two patients and the annual FVC decline rate decreased in another patient after nintedanib initiation. Consolidation with traction bronchiectasis and honeycombing on chest computed tomography (CT) suggested improved FVC change and no improvement, respectively. Nintedanib was discontinued in one patient because of death. Diarrhea was observed in two patients. CONCLUSIONS: Our exploratory study yielded two hypotheses; a potential benefit of nintedanib on the FVC annual decline and a possible role for chest CT findings predicting nintedanib efficacy. Unexpected adverse events were not observed.