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Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study

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Abstract

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.

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Acknowledgments

We thank Dr. Cordula Fassbender and Prof. Reinhard Klingel for helpful comments on the manuscript.

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    Authors and Affiliations

    1. Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany

      Josephine Heine, Lam-Thanh Ly, Carsten Finke, Harald Prüss & Lutz Harms

    2. German Center for Neurodegenerative Diseases (DZNE) Berlin, Charitéplatz 1, 10117, Berlin, Germany

      Lam-Thanh Ly & Harald Prüss

    3. Department of Nephrology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany

      Ina Lieker & Torsten Slowinski

    4. Berlin School of Mind and Brain, Humboldt-Universität zu Berlin, Luisenstr. 56, 10117, Berlin, Germany

      Carsten Finke

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    1. Josephine Heine
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    Correspondence to Josephine Heine.

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    Ethical standards

    All participants or next of kin gave informed written consent for participation. The study was approved by the Ethics Committee of the Charité University Hospital Berlin and conducted in accordance with the principles of the Declaration of Helsinki (1964) and its later amendments.

    Conflicts of interests

    Dr. Slowinski reports Grants from DIAMED, Germany, during the conduct of the study; Grants from Fresenius Medical Care, Germany, outside the submitted work. Dr. Prüss reports Grants from AFI, during the conduct of the study. Dr. Harms reports speaker honoraria from Biogen, Bayer, Genzyme, Roche, Novartis, Grifols and Merck-Serono. He serves on the advisory board for Roche, Biogen, Novartis, Genzyme and TEVA and has received travel support from Bayer, Grifols, Novartis and Biogen; outside of the submitted work.

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    JH and LTL contributed equally to the work.

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    Heine, J., Ly, LT., Lieker, I. et al. Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study. J Neurol 263, 2395–2402 (2016). https://doi.org/10.1007/s00415-016-8277-y

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    • DOI: https://doi.org/10.1007/s00415-016-8277-y

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