Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study.
Therapeutic apheresis has emerged as a major treatment option for autoantibody-associated inflammatory diseases of the nervous system. This includes patients with autoimmune encephalitides caused by antibodies against neuronal proteins. Plasma exchange (PE) and immunoadsorption (IA) constitute two possibilities to eliminate pathogenic antibodies from patients' plasma, but their efficacy and safety has not been prospectively assessed in larger patient groups of autoimmune encephalitides. In a prospective observational case control study, we, therefore, investigated the disease courses and treatment effects of 21 patients with autoimmune encephalitis associated with NMDAR, LGI1, CASPR2, GAD, mGluR5 and Hu antibodies. Patients were randomly assigned to receive PE (n = 11) or IA (n = 10). Symptoms were evaluated using the modified Rankin Scale (mRS). Side effects or adverse events were recorded. Both interventions, IA (p = 0.014) and PE (p = 0.01), resulted in significant reduction of the median mRS. With IA, 60 % of the patients improved clinically by at least 1 mRS score, none worsened. PE led to a comparable symptom reduction in 67 % of the cases. During 83 PE sessions, three adverse events were documented, while no side effects occurred under IA. Symptom improvement was significantly associated with younger age (r = -0.58), but not with disease duration. Therapeutic apheresis was most effective for neuronal surface antigens (83.3 %), followed by intracellular-synaptic antigens (66.7 %). Both IA and PE resulted in moderate to marked clinical improvement, with a low rate of adverse events. Apheresis is well tolerated and effective also as first-line therapy in autoimmune encephalitis, particularly in patients with antibodies targeting neuronal surfaces.
Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany. josephine.heine@charite.de.
Josephine
http://orcid.org/0000-0001-5226-6650
J
Heine
Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.|German Center for Neurodegenerative Diseases (DZNE) Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Lam-Thanh
LT
Ly
Department of Nephrology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Ina
I
Lieker
Department of Nephrology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Torsten
T
Slowinski
Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.|Berlin School of Mind and Brain, Humboldt-Universität zu Berlin, Luisenstr. 56, 10117, Berlin, Germany.
Carsten
C
Finke
Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.|German Center for Neurodegenerative Diseases (DZNE) Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Harald
H
Prüss
Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Lutz
L
Harms
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000906:Antibodies / Q000378:metabolism; D051959:ELAV Proteins / Q000276:immunology; D004660:Encephalitis / Q000276:immunology* / Q000628:therapy*; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D050031:Hashimoto Disease / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D007163:Immunosorbent Techniques; D007167:Immunotherapy / Q000379:methods*; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008565:Membrane Proteins / Q000276:immunology; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology; D010865:Pilot Projects; D010951:Plasma Exchange / Q000379:methods*; D011506:Proteins / Q000276:immunology; D064529:Receptor, Metabotropic Glutamate 5 / Q000276:immunology; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
D000906:Antibodies; C403049:CNTNAP2 protein, human; D051959:ELAV Proteins; C578935:GRM5 protein, human; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D011506:Proteins; D064529:Receptor, Metabotropic Glutamate 5; D016194:Receptors, N-Methyl-D-Aspartate; D005968:Glutamate Decarboxylase
Autoimmune encephalitis; Autoimmunity; Immunoadsorption; Immunotherapy; Plasma exchange