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Abstract Number: 2476

Blinatumomab in rapid progressive systemic sclerosis

Christina Gebhardt1, Franziska Szelinski2, Hector Rincon-Arevalo2, Giulia Magno3, Veit Buecklein3, Gerulf Haenel4, Gerhard Zugmaier5, Michael von Bergwelt3, Marion Subklewe3, Thomas Dörner6, Alla Skapenko7 and Hendrik Schulze-Koops7, 1LMU Hospital, Division for Rheumatology and Clinical Immunology, München, Germany, 2Charite Universitétsmedizin Berlin, Germany, Berlin, Germany, 3LMU Klinikum, Med. Klinik und Poliklinik III, Munich, Germany, 4LMU Gene Center, Munich, Germany, 5Amgen, Munich, Germany, 6Charite Universitétsmedizin Berlin, Germany and DRFZ, Berlin, Berlin, Germany, 7LMU Hospital, Division for Rheumatology and Clinical Immunology, Munich, Bayern, Germany

Meeting: ACR Convergence 2025

Keywords: B-Cell Targets, Cohort Study, Fibrosing syndromes, Scleroderma, Systemic, T-Lymphocyte

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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.


Disclosures: C. Gebhardt: AbbVie/Abbott, 6, Bristol-Myers Squibb(BMS), 6, Eli Lilly, 2, 6, Janssen, 6, UCB, 1, 6; F. Szelinski: None; H. Rincon-Arevalo: None; G. Magno: None; V. Buecklein: None; G. Haenel: None; G. Zugmaier: Amgen, 3; M. von Bergwelt: None; M. Subklewe: None; T. Dörner: AbbVie, 5, 12, Consulting fees, Celgene, 5, 12, Consulting fees, Deutsche Forschungsgemeinschaft, 5, Eli Lilly, 5, 12, Consulting fees, EMD Merck Serono, 5, 12, Consulting fees, EU Horizon2020 Harmonic SS, 5, Gilead/Galapagos, 12, Consulting fees, GlaxoSmithKlein(GSK), 5, 12, , EMD Merck Serono, Janssen, 5, 12, EMD Merck Serono, Novartis, 5, 12, , EMD Merck Serono, Roche, 5, 12, EMD Merck Serono, Sanofi, 5, UCB, 5; A. Skapenko: None; H. Schulze-Koops: AbbVie, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution), Amgen, 12, Research support and principal investigator (clinical trials funds to institution), Biogen Idec, 12, Research support and principal investigator (clinical trials funds to institution), Boehringer Ingelheim, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution), Bristol Myers Squibb, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution), Eli Lilly, 6, 12, Research support and principal investigator (clinical trials funds to institution), Galapagos, 2, GSK, 12, Research support and principal investigator (clinical trials funds to institution), Janssen, 6, Janssen-Cilag, 12, Research support and principal investigator (clinical trials funds to institution), Merck Sharp & Dohme, 6, 12, Research support and principal investigator (clinical trials funds to institution), Novartis, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution), Pfizer, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution), Roche, 12, Research support and principal investigator (clinical trials funds to institution), Sandoz-Hexal, 6, 12, Research support and principal investigator (clinical trials funds to institution), Sanofi, 6, 12, Research support and principal investigator (clinical trials funds to institution), UCB, 2, 6, 12, Research support and principal investigator (clinical trials funds to institution).

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 .
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