Neurology(R) neuroimmunology & neuroinflammation · 2026
MOG Antibody Status Is Associated With Distinct Clinical Profiles and Treatment Responses in Chronic Relapsing Inflammatory Optic Neuropathy.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод пока не подготовлен
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Graure Madalina, Nierobisch Nathalie, De Vere-Tyndall Anthony, Pakeerathan Thivya, Ayzenberg Ilya, Gernert Jonathan A, Havla Joachim, Ringelstein Marius, Aktas Orhan, Tkachenko Daria, Hümmert Martin Werner, Trebst Corinna, Cerdá-Fuertes Nuria, Papadopoulou Athina, Giglhuber Katrin, Wicklein Rebecca, Berthele Achim, Weller Michael, Kana Veronika, Roth Patrick, Herwerth Marina
Аннотация
BACKGROUND AND OBJECTIVES: Chronic relapsing inflammatory optic neuropathy (CRION) is a steroid-dependent form of optic neuritis with incompletely understood pathophysiology. The identification of myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) in a substantial patient subset has challenged the diagnostic and therapeutic management. The aim of this study was to investigate clinical profiles and treatment outcomes of patients with CRION, comparing MOG-IgG-positive (MOG+) and seronegative (MOG-) subgroups.
METHODS: Patients from 6 European tertiary centers fulfilling diagnostic criteria for CRION were included. All underwent cell-based autoantibody testing. Clinical outcomes (visual acuity and annualized relapse rate), laboratory and imaging findings (MRI, OCT), and treatment responses were retrospectively analyzed.
RESULTS: Sixty patients were included (median age 33 years; 70% female); 27 (45%) were MOG+. MOG+ CRION was associated with later onset, higher annualized relapse rate (ARR) before treatment (median [IQR] 2 [1-3] vs 1 [1-2], p = 0.023), and a trend toward shorter interrelapse intervals. Additional distinguishing features included higher frequencies of antinuclear antibody positivity, elevated CSF interleukin-6, and extensive enhancement of the optic nerve on MRI. Relapse burden correlated with visual acuity decline and retinal thinning. In MOG+ patients, monoclonal antibody therapy reduced the ARR (n = 21; 2 [1-3] vs 0 [0-2], p = 0.024), primarily driven by tocilizumab (n = 11; 2 [1-3] vs 0 [0-1], p = 0.023). In MOG- patients, rituximab and azathioprine showed a trend toward ARR reduction.
DISCUSSION: CRION represents a heterogeneous syndrome encompassing distinct subgroups. Although MOG+ patients demonstrate higher disease activity, tocilizumab might represent a therapeutic option, though further validation is needed. Serologic testing is critical for treatment stratification and preventing relapses.
METHODS: Patients from 6 European tertiary centers fulfilling diagnostic criteria for CRION were included. All underwent cell-based autoantibody testing. Clinical outcomes (visual acuity and annualized relapse rate), laboratory and imaging findings (MRI, OCT), and treatment responses were retrospectively analyzed.
RESULTS: Sixty patients were included (median age 33 years; 70% female); 27 (45%) were MOG+. MOG+ CRION was associated with later onset, higher annualized relapse rate (ARR) before treatment (median [IQR] 2 [1-3] vs 1 [1-2], p = 0.023), and a trend toward shorter interrelapse intervals. Additional distinguishing features included higher frequencies of antinuclear antibody positivity, elevated CSF interleukin-6, and extensive enhancement of the optic nerve on MRI. Relapse burden correlated with visual acuity decline and retinal thinning. In MOG+ patients, monoclonal antibody therapy reduced the ARR (n = 21; 2 [1-3] vs 0 [0-2], p = 0.024), primarily driven by tocilizumab (n = 11; 2 [1-3] vs 0 [0-1], p = 0.023). In MOG- patients, rituximab and azathioprine showed a trend toward ARR reduction.
DISCUSSION: CRION represents a heterogeneous syndrome encompassing distinct subgroups. Although MOG+ patients demonstrate higher disease activity, tocilizumab might represent a therapeutic option, though further validation is needed. Serologic testing is critical for treatment stratification and preventing relapses.