Time to Disability Milestones and Annualized Relapse Rates in NMOSD and MOGAD.
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Duchow Ankelien, Bellmann-Strobl Judith, Friede Tim, Aktas Orhan, Angstwurm Klemens, Ayzenberg Ilya, Berthele Achim, Dawin Eva, Engels Daniel, Fischer Katinka, Flaskamp Martina, Giglhuber Katrin, Grothe Matthias, Havla Joachim, Hümmert Martin W, Jarius Sven, Kaste Matthias, Kern Peter, Kleiter Ingo, Klotz Luisa, Korporal-Kuhnke Mirjam, Kraemer Markus, Krumbholz Markus, Kümpfel Tania, Lohmann Lisa, Ringelstein Marius, Rommer Paulus, Schindler Patrick, Schubert Charlotte, Schwake Carolin, Senel Makbule, Then Bergh Florian, Tkachenko Daria, Tumani Hayrettin, Trebst Corinna, Vardakas Ioannis, Walter Annette, Warnke Clemens, Weber Martin S, Wickel Jonathan, Wildemann Brigitte, Winkelmann Alexander, Paul Friedemann, Stellmann Jan-Patrick, Häußler Vivien, Neuromyelitis Optica Study Group (NEMOS)
Аннотация
METHODS: We analyzed data from individuals with NMOSD and MOGAD from the German Neuromyelitis Optica Study Group registry. Applying survival analyses, we estimated risk factors and computed time to disability milestones as defined by the Expanded Disability Status Score (EDSS).
RESULTS: We included 483 patients: 298 AQP4-IgG+ NMOSD, 52 AQP4-IgG-/MOG-IgG- NMOSD patients, and 133 patients with MOGAD. Despite comparable annualized attack rates, disability milestones occurred earlier and after less attacks in NMOSD patients than MOGAD patients (median time to EDSS 3: AQP4-IgG+ NMOSD 7.7 (95% CI 6.6-9.6) years, AQP4-IgG-/MOG-IgG- NMOSD 8.7) years, MOGAD 14.1 (95% CI 10.4-27.6) years; EDSS 4: 11.9 (95% CI 9.7-14.7), 11.6 (95% lower CI 7.6) and 20.4 (95% lower CI 14.1) years; EDSS 6: 20.1 (95% CI 16.5-32.1), 20.7 (95% lower CI 11.6), and 37.3 (95% lower CI 29.4) years; and EDSS 7: 34.2 (95% lower CI 31.1) for AQP4-IgG+ NMOSD). Higher age at onset increased the risk for all disability milestones, while risk of disability decreased over time.
INTERPRETATION: AQP4-IgG+ NMOSD, AQP4-IgG-/MOG-IgG- NMOSD, and MOGAD patients show distinctive relapse-associated disability progression, with MOGAD having a less severe disease course. Investigator-initiated research has led to increasing awareness and improved treatment strategies appearing to ameliorate disease outcomes for NMOSD and MOGAD. ANN NEUROL 2024;95:720-732.