Множественный склероз педиатрии Onset (POMS): изучение фенотипичной характеристики и пюбертального влияния в модуляции деятельности по болезням из Датского регистра MS (DMSR).
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Pilotto Silvy, Pontieri Luigi, Nielsen Helle Hvilsted, Rasmussen Peter Vestergaard, Svendsen Kristina Bacher, Marie Jensen Rikke, Blinkenberg Morten, Thormann Anja, Cocco Eleonora, Pugliatti Maura, Magyari Melinda
Аннотация
Оригинал публикации
Pediatric Onset Multiple Sclerosis (POMS): Exploring phenotypic characterization and pubertal influences in modulating the disease activity from the Danish MS Registry (DMSR).
BACKGROUND: Pediatric-onset multiple sclerosis (POMS) constitutes ~5% of multiple sclerosis (MS) cases and presents distinct clinical and diagnostic challenges. Puberty, characterized by significant hormonal changes, may influence disease presentation, relapse rates, and long-term outcomes.
OBJECTIVES: To investigate the impact of pubertal stages on clinical characteristics, relapse activity and disability progression in POMS using data from the Danish MS Registry (DMSR).
METHODS: A nationwide cohort of 185 POMS patients were included and categorized by pre- (<11 years), peri- (11-14 years), and post-pubertal (>14 years) onset. Demographics, presenting symptoms, magnetic resonance imaging (MRI) findings, relapse rates, and Expanded Disability Status Scale (EDSS) scores were compared. Patients transitioning across the three pubertal stages (n = 54) were analyzed longitudinally for relapse rate.
RESULTS: Pre-pubertal onset was associated with severe symptoms (cerebellar involvement, p = 0.042), greater lesion burden, higher 10-year disability (EDSS median = 3.75, p = 0.039), and lower relapse rates (annualized relapse rate (ARR) = 0.200). Male sex reduced relapse rates (p = 0.013). Female-to-male ratio increased from 1:1 pre-puberty to ~2:1 after puberty. Patients with pre-pubertal onset transitioning to peri- or post-puberty showed increasing relapse rates, peaking during peri-puberty (ARR = 0.302).
CONCLUSIONS: Puberty significantly modulates disease course in POMS, emphasizing the need for early, sex-specific interventions, proactive monitoring, and further exploration of hormonal influences on disease progression and treatment response.