AQP4-IgG autoimmunity in Japan and Germany: Differences in clinical profiles and prognosis in seropositive neuromyelitis optica spectrum disorders

Susanna Asseyer; Hiroki Masuda; Masahiro Mori; Judith Bellmann-Strobl; Klemens Ruprecht; Nadja Siebert; Graham Cooper; Claudia Chien; Ankelien Duchow; Jana Schließeit; Jia Liu; Kazuo Sugimoto; Akiyuki Uzawa; Ryohei Ohtani; Friedemann Paul; Alexander U Brandt; Satoshi Kuwabara; Hanna G Zimmermann

doi:10.1177/20552173211006862

AQP4-IgG autoimmunity in Japan and Germany: Differences in clinical profiles and prognosis in seropositive neuromyelitis optica spectrum disorders

Mult Scler J Exp Transl Clin. 2021 May 4;7(2):20552173211006862. doi: 10.1177/20552173211006862. eCollection 2021 Apr-Jun.

Authors

Susanna Asseyer¹, Hiroki Masuda², Masahiro Mori², Judith Bellmann-Strobl³, Klemens Ruprecht⁴, Nadja Siebert³, Graham Cooper³, Claudia Chien⁵, Ankelien Duchow⁵, Jana Schließeit⁵, Jia Liu², Kazuo Sugimoto², Akiyuki Uzawa², Ryohei Ohtani², Friedemann Paul⁵, Alexander U Brandt⁵, Satoshi Kuwabara², Hanna G Zimmermann⁵

Affiliations

¹ Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine and Charite-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany NeuroCure Clinical Research Center, Charite- Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
² Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
³ Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine, and Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
⁴ Department of Neurology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
⁵ Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine, and Charite - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.

Abstract

Background: Clinical outcomes in neuromyelitis optica spectrum disorders (NMOSD) vary across different regions.

Objective: To describe clinical profiles in Japanese and German NMOSD patients.

Methods: Medical records of aquaporin-4-immunoglobulin G (AQP4-IgG) positive NMOSD patients from Japan (n = 54) and Germany (n = 38) were retrospectively analyzed.

Results: The disability status was similar between both cohorts, although Japanese patients had a longer disease duration (13.3 ± 11.1 vs. 8.1 ± 6.9 years, p = 0.018) but similar relapse rates. Optic neuritis and myelitis were the most frequent attacks in both cohorts. Brain attacks occurred more frequently in Japanese patients (40.7% vs. 15.8%, p = 0.020). The time from disease onset (median [interquartile range] 2.3 [0.3-10.1] vs. 0.6 [0.2-1.9] years, p = 0.009) and the number of attacks (2.5 [1-7] vs. 2 [1-3], p = 0.047) until start of the first immunotherapy were higher in the Japanese cohort. Rituximab was the most common drug in the German cohort (52.6%) and not given in the Japanese cohort (p < 0.001), where oral prednisolone was the most common drug (92.6% vs. 15.8%, p < 0.001). The frequency of autoimmune comorbidities was higher in the German cohort (39.5% vs. 18.5%, p = 0.047).

Conclusion: Compared with Japanese NMOSD patients, German patients presented with similar disability despite shorter disease duration and earlier and more frequent immunosuppressive therapy.

Keywords: Anti-aquaporin 4 antibodies; autoimmune diseases; ethnicity; immunotherapy; neuromyelitis optica spectrum disorders.