Induction of stable, GC-free remission in patients with severe, therapy-refractory anti-synthetase syndrome after using the bispecific CD19xCD3 T cell engager blinatumomab

Christina Duesing¹, Ayla Nadja Stuetz², Andrea-Hermina Györfi³, Laura-Marie Lahu⁴, Franca Sophie Deicher⁵, Gamal Chehab⁶, Jutta Richter⁷, Marie Celine van Saan⁸, Bilgesu Safak Tümerdem², Alexandru-Emil Matei⁹, Bjoern Buehring¹⁰, Ricardo Grieshaber-Bouyer¹¹, Melanie Hagen¹², Georg Schett¹³, Wolfgang Merkt¹⁴ and Jörg Distler¹⁵, ¹Klinik für Rheumatologie, Düsseldorf, Germany, ²Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Nordrhein-Westfalen, Germany, ³Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University., Düsseldorf, Germany, ⁴Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany, ⁵Uniklinik Düsseldorf, Düsseldorf, Germany, ⁶Policlinic of Rheumatology and Hiller Research Unit Rheumatology, Heinrich-Heine-University, Düsseldorf, Germany, ⁷Clinic for Rheumatology and Hiller Research Unit, Heinrich-Heine-University Duesseldorf, Medical Faculty, Duesseldorf, Germany, Düsseldorf, Germany, ⁸Department of Rheumatology, University Hospital Duesseldorf, Medical Faculty of Heinrich Heine University, Duesseldorf, ⁹Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer Cluster of Excellence for Immune Mediated Diseases CIMD, Frankfurt am Main, Germany, Düsseldorf, Germany, ¹⁰Bergisches Rheuma-Zentrum, Wuppertal, Germany, ¹¹University Hospital Erlangen, Erlangen, Germany, ¹²Friedrich-Alexander-University Erlangen-Nuremberg, Department of Internal Medicine ³ - Rheumatology and Immunology, Erlangen, Germany, ¹³Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ¹⁴University Hospital Düsseldorf, Düsseldorf, Germany, ¹⁵University Hospital Duesseldorf and HHU, Duesseldorf, Germany

Meeting: ACR Convergence 2025

Keywords: B-Cell Targets, Myositis

Session Information

Date: Monday, October 27, 2025

Title: (1191–1220) Muscle Biology, Myositis & Myopathies – Basic & Clinical Science Poster II

Session Type: Poster Session B

Session Time: 10:30AM-12:30PM

Background/Purpose: Treatment of anti-synthetase syndrome (ASyS) presents clinical challenges: myositis can lead to permanent disability and severe organ involvement is life-threatening.

Methods: We treated three patients with multidrug-resistant severe anti-Jo-1 positive ASyS with blinatumomab 143µg and added rituximab (RTX) 1000mg for maintenance therapy. Patient 1, 67-year-old female, had reoccurring myositis and new interstitial lung disease (ILD). Patient 2, 36-year-old male, suffered from progressive ILD and myocarditis. Patient 3, 47-year-old male, had debilitating myositis. All patients were unresponsive to at least three immunosuppressants including rituximab.

Results: Blinatumomab induced complete depletion of CD19+ B cells in circulation and muscle tissue (Figure 2) and profound decreases in anti-Jo-1 titers. Blinatumomab induced glucocorticoid-free remission with normalization of muscle enzymes, improvement of muscle strength (Figure 1) in all patients. Patient 1 showed an increase in CD19+ B cells and recurrence of myositis 4 months after blinatumomab. We re-started blinatumomab plus RTX and reinduced clinical remission followed by maintenance therapy with RTX (follow-up six months). Patient 2 showed ongoing remission of myositis and improvement of ILD for six months after blinatumomab and repeated RTX. Patient 3 demonstrated ongoing remission for 6 months after blinatumomab and RTX.Adverse events: cytokine release syndrome (CRS) grade 3 (same day recovery) and respiratory infection in patient 3. No neurotoxicity occurred.

Conclusion: This is the first report on treating patients with severe, treatment-refractory ASyS with blinatumomab. When followed by RTX, glucocorticoid-free remission remained stable for up to 10 months follow-up. Combining blinatumomab with RTX may offer potential in treatment-refractory ASyS patients.

Disclosures: C. Duesing: None; A. Stuetz: None; A. Györfi: AbbVie, 6, Boehringer-Ingelheim, 6; L. Lahu: None; F. Deicher: None; G. Chehab: None; J. Richter: None; M. van Saan: None; B. Tümerdem: None; A. Matei: None; B. Buehring: None; R. Grieshaber-Bouyer: AstraZeneca, 1, Candid Therapeutics, 1, Cullinan Therapeutics, 1, Eli Lilly, 6; M. Hagen: None; G. Schett: Cabaletta, 6, Eli Lilly, 6, Janssen, 6, Kyverna, 6, Novartis, 6, UCB, 6; W. Merkt: Boehringer-Ingelheim, 6, Bristol-Myers Squibb(BMS), 5, Evotec, 5, Glapagos, 12, support for congress travel, Kyverna, 6, Lilly, 12, support for congress travel; J. Distler: 4D Science, 8, 11, Actelion, 2, 6, Active Biotech, 2, 6, Anamar, 2, 6, Array Biopharma, 2, 6, ARXX Therapeutics, 2, 6, aTyr Pharma, 2, 6, Bayer Pharma, 2, 6, BMS (Bristol-Myers Squibb), 2, 6, Boehringer Ingelheim, 2, 6, Celgene, 2, 6, FibroCure, 4, Galapagos, 2, 6, GSK, 2, 6, Inventiva, 2, 6, JB Therapeutics, 2, 6, Medac, 2, 6, Novartis, 2, 6, Pfizer, 2, 6, Redx Pharma, 2, 6, RuiYi, 2, 6, Sanofi-Aventis, 2, 6, UCB, 2, 6.

To cite this abstract in AMA style:

Duesing C, Stuetz A, Györfi A, Lahu L, Deicher F, Chehab G, Richter J, van Saan M, Tümerdem B, Matei A, Buehring B, Grieshaber-Bouyer R, Hagen M, Schett G, Merkt W, Distler J. Induction of stable, GC-free remission in patients with severe, therapy-refractory anti-synthetase syndrome after using the bispecific CD19xCD3 T cell engager blinatumomab [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/induction-of-stable-gc-free-remission-in-patients-with-severe-therapy-refractory-anti-synthetase-syndrome-after-using-the-bispecific-cd19xcd3-t-cell-engager-blinatumomab/. Accessed .

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