{
  "abstract": "Introduction Systemic sclerosis (SSc) is associated with the highest case-related mortality of all rheumatic diseases. B cell-targeting has shown efficacy in SSc but failed to eliminate the anti-topoisomerase-I autoantibody-producing plasma cell niches.Material and Methods We treated five cases of severe, progressive, treatment-refractory SSc with the plasma cell-depleting bispecific antibody teclistamab ( table 1). After six cycles of teclistamab, 1g of rituximab was administered to prevent recovery of autoantibody-producing cells. Four patients were positive for anti-topoisomerase-I antibodies, and one patient was positive for anti-fibrillarin autoantibodies. All patients had diffuse cutaneous SSc, interstitial lung disease and primary heart involvement and have been refractory to at least three immunomodulatory and/or antifibrotic therapies. Immunosuppressive and antifibrotic therapies were discontinued prior to baseline (BL). Patients were followed for up to 15 months.Results Treatment with teclistamab depleted all plasma cells in the skin. Anti-topoisomerase-I-autoantibody titers seroconverted in two patients and dropped by 83% and 33% in the third and fourth patient, respectively. This immunological response was associated with strong decreases of the modified Rodnan skin score, stabilization of SSc-ILD, resolution of tendon friction rubs and decreases in the EUSTAR-AI in all patients. The effect on SSc-associated primary myocardial involvement was heterogeneous. Treatment with teclistamab was associated with several adverse events including cytokine release syndrome, hypogammaglobulinemia and mild infections (despite regular immunoglobulin replacement).Conclusions Teclistamab may offer potential as a rescue therapy for selected patients suffering from severe, treatment-refractory dcSSc, including those with advanced disease.Abstract OC.52 Figure 1Abstract OC.52 Figure 2Abstract OC.52 Table 1",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer CIMD, Frankfurt am Main, Germany"
      ],
      "name": "Christina Düsing"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer CIMD, Frankfurt am Main, Germany"
      ],
      "name": "Andrea-Hermina Györfi"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Ayla Nadja Stütz"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Laura Marie Lahu"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Franca Sophie Deicher"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Yi-Nan Li"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer CIMD, Frankfurt am Main, Germany"
      ],
      "name": "Alexandru-Emil Matei"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Alexandru Micu"
    },
    {
      "affiliations": [
        "Department of Internal Medicine 3, Rheumatology and Clinical Immunology, FAU Erlangen-Nürnberg and UKE, Erlangen, Germany",
        "Deutsches Zentrum Immuntherapie (DZI), Friedrich-Alexander University (FAU) Erlangen-Nürnberg and UKE, Erlangen, Germany"
      ],
      "name": "Georg Schett"
    },
    {
      "affiliations": [
        "Department of Hematology and Oncology, University Hospital Düsseldorf and Heinrich-Heine University, Düsseldorf, Germany"
      ],
      "name": "Sascha Dietrich"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital Heidelberg, Heidelberg, Germany"
      ],
      "name": "Wolfgang Merkt"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany",
        "Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer CIMD, Frankfurt am Main, Germany"
      ],
      "name": "Jörg HW Distler"
    }
  ],
  "title": "OC.52 Targeting the plasma cell niche in systemic sclerosis: a case series about the bispecific anti-bCMAxCD3 antibody teclistamab in severe, treatment-refractory patients",
  "uid": "8a87f3ed-1260-570f-a1f2-e9eefeae71b8"
}