{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T15:33:58Z","timestamp":1792856038217,"version":"4.5.0"},"reference-count":17,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,2,10]],"date-time":"2022-02-10T00:00:00Z","timestamp":1644451200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100014364","name":"National Children's Research Centre","doi-asserted-by":"publisher","award":["EXC 2049\u201089829218"],"award-info":[{"award-number":["EXC 2049\u201089829218"]}],"id":[{"id":"10.13039\/100014364","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>A fraction of patients with antibody\u2010mediated autoimmune diseases remain unresponsive to first\u2010\/second\u2010line 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\u2010depleting anti\u2010CD38 antibody daratumumab in life\u2010threatening, antibody\u2010mediated autoimmune diseases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this retrospective, single\u2010center case series, seven patients with autoantibody\u2010driven neurological autoimmune diseases (autoimmune encephalitis, <jats:italic>n<\/jats:italic>\u00a0=\u00a05; neurofascin antibody\u2010associated chronic inflammatory demyelinating polyneuropathy associated with sporadic late onset nemaline myopathy, <jats:italic>n<\/jats:italic>\u00a0=\u00a01; seronegative myasthenia gravis, <jats:italic>n<\/jats:italic>\u00a0=\u00a01) unresponsive to a median of four (range = 4\u20139) immunotherapies were treated with four to 20 cycles of 16\u00a0mg\/kg daratumumab.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Daratumumab allowed a substantial clinical improvement in all patients, as measured by modified Rankin Scale (mRS; before treatment: mRS\u00a0=5, <jats:italic>n<\/jats:italic>\u00a0=\u00a07; after treatment: median mRS\u00a0=4, range = 0\u20135), Clinical Assessment Scale in Autoimmune Encephalitis (from median 21 to 3 points, <jats:italic>n<\/jats:italic>\u00a0=\u00a05), Inflammatory Neuropathy Cause and Treatment disability score (from 7 to 0 points, <jats:italic>n<\/jats:italic>\u00a0=\u00a01), and Quantitative Myasthenia Gravis score (from 16 to 8 points, <jats:italic>n<\/jats:italic>\u00a0=\u00a01). Daratumumab induced a substantial reduction of disease\u2010specific autoreactive antibodies, total IgG (serum, 66%, <jats:italic>n<\/jats:italic>\u00a0=\u00a07; cerebrospinal fluid, 58%, <jats:italic>n<\/jats:italic>\u00a0=\u00a05), and vaccine\u2010induced titers for rubella (50%) and tetanus toxoid (74%). Treatment\u2010related toxicities Grade 3 or higher occurred in five patients, including one death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our findings suggest that daratumumab provided a clinically relevant depletion of autoreactive long\u2010lived plasma cells, identifying plasma cell\u2010targeted therapies as promising escalation therapy for highly active, otherwise treatment\u2010refractory autoantibody\u2010mediated neurological diseases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15266","type":"journal-article","created":{"date-parts":[[2022,1,31]],"date-time":"2022-01-31T08:51:08Z","timestamp":1643619068000},"page":"1847-1854","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":88,"title":["Daratumumab for treatment\u2010refractory antibody\u2010mediated diseases in neurology"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8773-6090","authenticated-orcid":false,"given":"Franziska","family":"Scheibe","sequence":"first","affiliation":[{"name":"Department of Neurology and Experimental Neurology Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin  Berlin Germany"},{"name":"NeuroCure Clinical Research Center Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3553-6406","authenticated-orcid":false,"given":"Lennard","family":"Ostendorf","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin  Berlin Germany"},{"name":"German Rheumatism Research Centre Berlin  Berlin Germany"},{"name":"Department of Nephrology and Medical Intensive Care Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin  Berlin 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