Efficacy and safety of telitacicept in patients with progressive interstitial lung disease associated with antisynthetase syndrome, rheumatoid arthritis, or Sjögren's syndrome: a prospective observational study.

backgroundInterstitial lung disease (ILD) is a severe complication of antisynthetase syndrome, rheumatoid arthritis, and Sjögren's syndrome, contributing significantly to patient mortality. Standard treatment options remain limited. This study evaluated the efficacy and safety of telitacicept, a dual-target inhibitor of B-lymphocyte stimulator and a proliferation-inducing ligand, in patients with these specific ILD subtypes.

methodsThis prospective observational study included 18 patients with ILD associated with antisynthetase syndrome (n = 5), rheumatoid arthritis (n = 5), or Sjögren's syndrome (n = 8). Participants either demonstrated resistance to standard immunosuppressive regimens or presented with other connective tissue diseases (CTDs) and complex multisystemic manifestations. All patients received subcutaneous telitacicept (160 mg weekly) combined with glucocorticoids. Primary outcomes were absolute changes in forced vital capacity percentage (FVC%) and diffusing capacity of the lungs for carbon monoxide percentage (DLCO%) at 24 weeks. Secondary outcomes included exercise capacity (6-minute walk test), radiographic changes (Warrick score), and disease activity indices. Comparisons between baseline and 24 weeks were performed using paired t-tests.

resultsAfter 24 weeks, lung function significantly improved across all groups. FVC% increased by 20.94% in antisynthetase syndrome-ILD, 10.83% in rheumatoid arthritis-ILD, and 12.19% in Sjögren's syndrome-ILD (all P < 0.05). Similarly, DLCO% improved by 30.61%, 8.61%, and 9.73%, respectively (all P < 0.05). Exercise capacity significantly increased, with 6-minute walk distances improving by over 20% in all subtypes. Radiographic assessment showed a significant reduction in lung lesions, with Warrick scores decreasing by 44.73%, 27.04%, and 30.12%, respectively. Systemic disease activity also decreased significantly, and patients successfully reduced their daily prednisone dose to 5-7.5 mg. No adverse reactions were observed during the study period.

conclusionsTelitacicept demonstrated significant clinical efficacy and a favorable safety profile in patients with ILD associated with antisynthetase syndrome, rheumatoid arthritis, and Sjögren's syndrome. These findings suggest telitacicept may be a promising therapeutic option for refractory or complex connective tissue disease-associated ILD.