Session Information
Date: Tuesday, October 28, 2025
Title: (2470–2503) Systemic Sclerosis & Related Disorders – Clinical Poster III
Session Type: Poster Session C
Session Time: 10:30AM-12:30PM
Background/Purpose: Systemic sclerosis is a severe, potentially fatal disease, characterized by progressive fibrosis of skin and internal organs. Blinatumomab is a bispecific CD3/CD19-T-cell engager (BiTe) approved for treatment of relapsing/refractory Philadelphia chromosome negative, CD19-positive B-precursor-acute lymphocytic leukemia.
Methods: After our first case of blinatumomab for acute progressive systemic sclerosis despite conventional therapy, we now report of our cohort of up to date four BiTe treated patients.
Results: All patients showed rapidly progressive skin fibrosis as well as internal organ involvement, namely myocardial fibrosis in our first patient and pulmonary fibrosis in the other three. We administered four cycles of blinatumomab in our first two patients and two cycles with higher daily dose in the other two patients due to good initial results and low grade toxicity. All patients reported of rapid improvement of skin symptoms with a decrease of the Rodnan Skin Score score from (range) 20 to 5 at our last follow-up. Furthermore we could demonstrate receding cardial as well as pulmonary fibrosis, as seen in imaging (cardiac MRI) and lung function tests (increase of DLCOs by up to 11 %). In all patients, peripheral B cell counts declined completely from the first day of treatment with recovery to normal levels within weeks after treatment. Peripheral T cells were significantly reduced on the first day of treatment, but quickly recovered to normal levels and remained stable during subsequent cycles. IgG and Scl 70 ab levels temporarily decreased after treatment. Remarkably, with the exception of a transient neutropenia in one of the four patients, there were no significant adverse events, in particular ICANs, CRS or infections.
Conclusion: Blinatumomab resulted in profound B-cell depletion and a significant decrease in serum IgG with no increased susceptibility to infections. Clinically, the therapy led to a rapid and sustained improvement in symptoms. Further studies are needed to determine the value of B-cell depleting therapy with blinatumomab in systemic sclerosis.
To cite this abstract in AMA style:
Gebhardt C, Szelinski F, Rincon-Arevalo H, Magno G, Buecklein V, Haenel G, Zugmaier G, von Bergwelt M, Subklewe M, Dörner T, Skapenko A, Schulze-Koops H. Blinatumomab in rapid progressive systemic sclerosis [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/blinatumomab-in-rapid-progressive-systemic-sclerosis/. Accessed .« Back to ACR Convergence 2025
ACR Meeting Abstracts - https://acrabstracts.org/abstract/blinatumomab-in-rapid-progressive-systemic-sclerosis/