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@ARTICLE{Scheibe:163492,
      author       = {Scheibe, Franziska and Ostendorf, Lennard and Prüss,
                      Harald and Radbruch, Helena and Aschman, Tom and Hoffmann,
                      Sarah and Blau, Igor-Wolfgang and Meisel, Christian and
                      Alexander, Tobias and Meisel, Andreas},
      title        = {{D}aratumumab for treatment-refractory antibody-mediated
                      diseases in neurology.},
      journal      = {European journal of neurology},
      volume       = {29},
      number       = {6},
      issn         = {1351-5101},
      address      = {Oxford},
      publisher    = {Blackwell Science},
      reportid     = {DZNE-2022-00252},
      pages        = {1847-1854},
      year         = {2022},
      note         = {ISSN 1468-1331 not unique: **2 hits**. (CC BY-NC-ND)},
      abstract     = {A fraction of patients with antibody-mediated autoimmune
                      diseases remain unresponsive to first-/second-line and
                      sometimes even to escalation immunotherapies. Because these
                      patients are still affected by poor outcome and increased
                      mortality, we investigated the safety and efficacy of the
                      plasma cell-depleting anti-CD38 antibody daratumumab in
                      life-threatening, antibody-mediated autoimmune diseases.In
                      this retrospective, single-center case series, seven
                      patients with autoantibody-driven neurological autoimmune
                      diseases (autoimmune encephalitis, n = 5; neurofascin
                      antibody-associated chronic inflammatory demyelinating
                      polyneuropathy associated with sporadic late onset nemaline
                      myopathy, n = 1; seronegative myasthenia gravis, n = 1)
                      unresponsive to a median of four (range = 4-9)
                      immunotherapies were treated with four to 20 cycles of 16
                      mg/kg daratumumab.Daratumumab allowed a substantial clinical
                      improvement in all patients, as measured by modified Rankin
                      Scale (mRS; before treatment: mRS =5, n = 7; after
                      treatment: median mRS =4, range = 0-5), Clinical Assessment
                      Scale in Autoimmune Encephalitis (from median 21 to 3
                      points, n = 5), Inflammatory Neuropathy Cause and Treatment
                      disability score (from 7 to 0 points, n = 1), and
                      Quantitative Myasthenia Gravis score (from 16 to 8 points, n
                      = 1). Daratumumab induced a substantial reduction of
                      disease-specific autoreactive antibodies, total IgG (serum,
                      $66\%,$ n = 7; cerebrospinal fluid, $58\%,$ n = 5), and
                      vaccine-induced titers for rubella $(50\%)$ and tetanus
                      toxoid $(74\%).$ Treatment-related toxicities Grade 3 or
                      higher occurred in five patients, including one death.Our
                      findings suggest that daratumumab provided a clinically
                      relevant depletion of autoreactive long-lived plasma cells,
                      identifying plasma cell-targeted therapies as promising
                      escalation therapy for highly active, otherwise
                      treatment-refractory autoantibody-mediated neurological
                      diseases.},
      keywords     = {Antibodies, Monoclonal / Autoantibodies / Encephalitis /
                      Hashimoto Disease / Humans / Myasthenia Gravis / Nervous
                      System Diseases: drug therapy / Neurology / Retrospective
                      Studies / CIDP (Other) / autoimmune encephalitis (Other) /
                      daratumumab (Other) / myasthenia gravis (Other) / sporadic
                      late onset nemaline myopathy (Other)},
      cin          = {AG Prüß},
      ddc          = {610},
      cid          = {I:(DE-2719)1810003},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:35098616},
      doi          = {10.1111/ene.15266},
      url          = {https://pub.dzne.de/record/163492},
}