%0 Journal Article
%A Scheibe, Franziska
%A Ostendorf, Lennard
%A Prüss, Harald
%A Radbruch, Helena
%A Aschman, Tom
%A Hoffmann, Sarah
%A Blau, Igor-Wolfgang
%A Meisel, Christian
%A Alexander, Tobias
%A Meisel, Andreas
%T Daratumumab for treatment-refractory antibody-mediated diseases in neurology.
%J European journal of neurology
%V 29
%N 6
%@ 1351-5101
%C Oxford
%I Blackwell Science
%M DZNE-2022-00252
%P 1847-1854
%D 2022
%Z ISSN 1468-1331 not unique: **2 hits**. (CC BY-NC-ND)
%X 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
%K Antibodies, Monoclonal
%K Autoantibodies
%K Encephalitis
%K Hashimoto Disease
%K Humans
%K Myasthenia Gravis
%K Nervous System Diseases: drug therapy
%K Neurology
%K Retrospective Studies
%K CIDP (Other)
%K autoimmune encephalitis (Other)
%K daratumumab (Other)
%K myasthenia gravis (Other)
%K sporadic late onset nemaline myopathy (Other)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:35098616
%R 10.1111/ene.15266
%U https://pub.dzne.de/record/163492