Factors Associated With Relapse and Treatment of Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease in the United Kingdom

Abstract

IMPORTANCE Longer-term outcomes and risk factors associated with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are not well established. OBJECTIVE To investigate longer-term risk of relapse and factors associated with this risk among patients with MOGAD. DESIGN, SETTING, AND PARTICIPANTS This large, single-nation, prospective cohort study was conducted among 276 patients with MOGAD at 5 health care centers in the UK. Data from January 1973 to March 2020 were collected from 146 patients at Oxford and its outreach sites, 65 patients at Liverpool, 32 patients at a children's hospital in Birmingham, 22 patients at a children's hospital in London, and 11 patients at Cardiff, Wales. Data were analyzed from April through July 2020.MAIN OUTCOMES AND MEASURES Risk of relapse and annualized relapse rate were evaluated according to different baseline features, including onset age, onset phenotype, and incident vs nonincident group, with the incident group defined as patients diagnosed with antibodies against myelin oligodendrocyte glycoprotein before a second attack. Time to next relapse among patients experiencing relapse was measured and compared between the maintenance therapy subgroup and each first-line treatment group. The no-treatment group was defined as the off-treatment phase among patients who were relapsing, which could occur between any attack or between the last attack and last follow-up. RESULTS Among 276 patients with MOGAD, 183 patients were identified as being part of the incident group. There were no differences in mean (SD) onset age between total and incident groups (26.4 [17.6] years vs 28.2 [18.1] years), and female patients were predominant in both groups (166 [60.1%] female patients vs 106 [57.9%] female patients). The most common presentation overall was optic neuritis (ON) (119 patients among 275 patients with presentation data [43.3%]), while acute disseminated encephalomyelitis (ADEM), brain, or brainstem onset was predominant among 69 patients aged younger than 12 years (47 patients [68.1%]), including 41 patients with ADEM (59.4%). In the incident group, the 8-year risk of relapse was 36.3% (95% CI, 27.1%-47.5%). ON at onset was associated with increased risk of relapse compared with transverse myelitis at onset (hazard ratio [HR], 2.66; 95% CI, 1.01-6.98; P = .047), but there was no statistically significant difference with adjustment for a follow-on course of corticosteroids. Any TM at onset (ie, alone or in combination with other presentations [ie, ON or ADEM, brain, or brain stem]) was associated with decreased risk of relapse compared with no TM (HR, 0.41; 95% CI, 0.20-0.88; P = .01). Young adult age (ie, ages >18-40 years) was associated with increased risk of relapse compared with older adult age (ie, ages >40 years) (HR, 2.71; 95% CI, 1.18-6.19; P = .02). First-line maintenance therapy was (continued) Key Points Question Which factors are associated with long-term risk of relapse in myelin oligodendrocyte glycoprotein antibodyassociated disease (MOGAD)? Findin...

Myelin oligodendrocyte glycoprotein antibody‐associated disease presenting as recurrent acute disseminated encephalomyelitis: Case report of the youngest Mexican patient in the literature

“…According to these results, the age of the present patient could have been a risk factor for further relapses. Of note, in that study, relapsing ADEM was found only in patients aged <12 years 9 . They also reported that the clinical phenotype might influence relapses; optic neuritis has a significant higher risk of disease activity compared with those with transverse myelitis (HR 2.66).…”

Meningo-cortical manifestations of myelin oligodendrocyte glycoprotein antibody-associated disease: Review of a novel clinico-radiographic spectrum

“…Expectedly, patients can have other attacks compatible with MOGAD prior to, concurrent with, or after unilateral cortical FLAMES/UCCE (12). Maintenance immunotherapy should be considered in patients with MOGAD who have relapsing disease, although the optimal long-term treatment approach remains unclear (52)(53)(54)(55).…”

Neuro-ophthalmological Presentation of Optic Neuritis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

“…In up to 20% of MOGAD patients, associations were found between a possible trigger and a first MOGAD event [3,8,[42][43][44][45]. Temporal associations have been reported with N-methyl-d-aspartate receptor encephalitis, infections, including herpes simplex virus, Borrelia, Epstein-Barr as well as, more recently, severe acute respiratory syndrome coronavirus type 2 (SARS-sCoV-2) [8,[42][43][44][45][46][47] and, albeit less frequently, with vaccinations (mostly with SARS-CoV-2 vaccination but also with diphtheria, tetanus, pertussis, polio, and influenza vaccination) [3,8,[42][43][44][45]. Recent research has shown that post-vaccination ON in the presence of MOG-IgG is particularly severe, with around 50% of affected patients experiencing severe and debilitating vision loss [45].…”

“…Long-term treatment is recommended for patients at risk for relapse and current therapies comprise the off-label use of prednisolone, steroid-sparing immunosuppression with azathioprine, methotrexate, mycophenolate mofetil, rituximab and IVIG [75][76][77][78][79]. Maintenance treatment is given either as monotherapy or as combination therapy [9,38,42]. Current data do not show any indication for a relapseindependent disease progression, but the course of symptoms including visual quality of life over time from the patients' perspective need further investigation.…”