TY  - JOUR
AU  - Scheibe, Franziska
AU  - Ostendorf, Lennard
AU  - Prüss, Harald
AU  - Radbruch, Helena
AU  - Aschman, Tom
AU  - Hoffmann, Sarah
AU  - Blau, Igor-Wolfgang
AU  - Meisel, Christian
AU  - Alexander, Tobias
AU  - Meisel, Andreas
TI  - Daratumumab for treatment-refractory antibody-mediated diseases in neurology.
JO  - European journal of neurology
VL  - 29
IS  - 6
SN  - 1351-5101
CY  - Oxford
PB  - Blackwell Science
M1  - DZNE-2022-00252
SP  - 1847-1854
PY  - 2022
N1  - ISSN 1468-1331 not unique: **2 hits**. (CC BY-NC-ND)
AB  - 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
KW  - Antibodies, Monoclonal
KW  - Autoantibodies
KW  - Encephalitis
KW  - Hashimoto Disease
KW  - Humans
KW  - Myasthenia Gravis
KW  - Nervous System Diseases: drug therapy
KW  - Neurology
KW  - Retrospective Studies
KW  - CIDP (Other)
KW  - autoimmune encephalitis (Other)
KW  - daratumumab (Other)
KW  - myasthenia gravis (Other)
KW  - sporadic late onset nemaline myopathy (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:35098616
DO  - DOI:10.1111/ene.15266
UR  - https://pub.dzne.de/record/163492
ER  -