Abstract
Sarcoidosis is a systemic inflammatory disease of unknown origin that can progress to fibrocavernous disease. Published data on antifibrotic therapy for fibrotic sarcoidosis are contradictory, largely due to an insufficient number of studies. In randomized trials, nintedanib has demonstrated efficacy in reducing the progression of interstitial lung disease; however, it has not shown a positive effect specifically in fibrotic sarcoidosis. Some individual studies have reported beneficial effects of antifibrotic therapy in cases of fibrotic sarcoidosis. Due to the limited and conflicting data, each observation of antifibrotic therapy in fibrotic sarcoidosis is valuable. The aim . Here, we describe our experience of using antifibrotic therapy in a patient with fibrotic pulmonary sarcoidosis. Methods . The patient, a 66-year-old woman with sarcoidosis-associated pulmonary fibrosis, has been receiving antifibrotic therapy (nintedanib 150 mg twice daily) since October 2022. Efficacy and safety were assessed before initiation and after 12, 24 and 32 months of antifibrotic therapy. Results . The respiratory deterioration slowed down, evidenced by an increased distance in the 6-minute walk test. Pulmonary function decline was also reduced, with no decrease in FEV 1 , FVC, FRC, or RV. Computed tomography scans revealed no progression in the volume of pulmonary fibrosis. Conclusion . The use of antifibrotic therapy was associated with stabilization of pulmonary function and no progression of pulmonary fibrosis. Further studies on antifibrotic therapy in fibrotic sarcoidosis are warranted.