RESUMEN
OBJECTIVE: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. METHODS: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. RESULTS: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. ON was the dominant phenotype (UON 35%, BON 19%) of all clinical episodes. 109/226 (48%) MRIs had no brain lesions. Patients were steroid responsive, but 70% of episodes treated with oral prednisone relapsed, particularly at doses <10 mg daily or within 2 months of cessation. Immunotherapy, including maintenance prednisone (P=0.0004), intravenous immunoglobulin, rituximab and mycophenolate, all reduced median ARRs on-treatment. Treatment failure rates were lower in patients on maintenance steroids (5%) compared with non-steroidal maintenance immunotherapy (38%) (P=0.016). 58% of patients experienced residual disability (average follow-up 61 months, visual loss in 24%). Patients with ON were less likely to have sustained disability defined by a final EDSS of ≥2 (OR 0.15, P=0.032), while those who had any myelitis were more likely to have sustained residual deficits (OR 3.56, P=0.077). CONCLUSION: Relapsing MOG antibody-associated demyelination is strongly associated with ON across all age groups and ADEM in children. Patients are highly responsive to steroids, but vulnerable to relapse on steroid reduction and cessation.
Asunto(s)
Enfermedades Autoinmunes Desmielinizantes SNC/terapia , Inmunosupresores/uso terapéutico , Adolescente , Adulto , Anciano , Autoanticuerpos/inmunología , Encéfalo/diagnóstico por imagen , Niño , Preescolar , Estudios de Cohortes , Enfermedades Autoinmunes Desmielinizantes SNC/diagnóstico por imagen , Enfermedades Autoinmunes Desmielinizantes SNC/inmunología , Enfermedades Autoinmunes Desmielinizantes SNC/fisiopatología , Encefalomielitis Aguda Diseminada/diagnóstico por imagen , Encefalomielitis Aguda Diseminada/inmunología , Encefalomielitis Aguda Diseminada/fisiopatología , Encefalomielitis Aguda Diseminada/terapia , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Inmunoterapia , Lactante , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Ácido Micofenólico/uso terapéutico , Glicoproteína Mielina-Oligodendrócito/inmunología , Mielitis Transversa/diagnóstico por imagen , Mielitis Transversa/inmunología , Mielitis Transversa/fisiopatología , Mielitis Transversa/terapia , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/inmunología , Neuromielitis Óptica/fisiopatología , Neuromielitis Óptica/terapia , Neuritis Óptica/diagnóstico por imagen , Neuritis Óptica/inmunología , Neuritis Óptica/fisiopatología , Neuritis Óptica/terapia , Prednisona/uso terapéutico , Rituximab/uso terapéutico , Adulto JovenRESUMEN
Fingolimod is an oral sphingosine-1-phosphate (S1P) receptor modulator and the first oral therapy for relapsing-remitting multiple sclerosis. Its use has been complicated by a low rate of cystoid macular edema usually in the first 3 months after commencement of the medication. We report the case of a 34-year-old male with relapsing-remitting multiple sclerosis, who developed acute anterior uveitis on day 5 of fingolimod treatment. He responded to appropriate treatment and cessation of drug, but developed low-grade chronic anterior uveitis without cystoid macular edema. We discuss possible mechanisms of uveitis onset in this group of patients. Urgent ophthalmological review is recommended for patients receiving fingolimod therapy who develop a red, painful eye, which may occur within 5 days of fingolimod treatment initiation.