Amorim Ana Luiza M, Cabral Nadia C, Osaku Fabiane M, Len Claudio A, Oliveira Enedina M L, Terreri Maria Teresa
Universidade Federal de São Paulo (Unifesp), Departamento de Pediatria, Setor de Reumatologia Pediátrica, São Paulo, SP, Brazil.
Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
Rev Bras Reumatol Engl Ed. 2017 May-Jun;57(3):224-228. doi: 10.1016/j.rbre.2016.09.016. Epub 2016 Oct 25.
Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population.
To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients.
22 patients younger than 21 years old with MS or NMO diagnosed before the age of 18 years were evaluated regarding epidemiological data, clinical presentation, association with autoimmune diseases, family history of autoimmune diseases, laboratory findings, imaging studies and presence of auto-antibodies.
Among the patients studied, there was a prevalence of females (68.1%). The mean age of symptoms onset was 8 years and 9 months and the mean current age was 16 years and 4 months. Two patients (9%) had a history of associated autoimmune rheumatic disease: one case of juvenile dermatomyositis in a patient with NMO and another of systemic lupus erythematosus in a patient with MS. Three patients (13%) had a family history of autoimmunity in first-degree relatives. Antinuclear antibody was found positive in 80% of patients with NMO and 52% of patients with MS. About 15% of antinuclear antibody-positive patients were diagnosed with rheumatologic autoimmune diseases.
Among patients with demyelinating diseases diagnosed in childhood included in this study there was a high frequency of antinuclear antibody positivity but a lower association with rheumatologic autoimmune diseases than that observed in studies conducted in adults.
多发性硬化症（MS）和视神经脊髓炎（NMO）是中枢神经系统的脱髓鞘疾病。脱髓鞘疾病患者及其家族中的自身免疫情况已得到广泛研究和讨论。近期研究表明，成年MS或NMO患者及其家族中风湿性自身免疫疾病的发病率较高，但儿科人群中尚无相关研究。
评估儿科患者中MS和NMO与自身免疫性风湿疾病的关联。
对22例年龄小于21岁、18岁前诊断为MS或NMO的患者进行了流行病学数据、临床表现、与自身免疫疾病的关联、自身免疫疾病家族史、实验室检查结果、影像学研究及自身抗体检测。
在研究的患者中，女性占比68.1%。症状出现的平均年龄为8岁9个月，当前平均年龄为16岁4个月。2例患者（9%）有相关自身免疫性风湿疾病史：1例NMO患者合并幼年皮肌炎，另1例MS患者合并系统性红斑狼疮。3例患者（13%）的一级亲属有自身免疫家族史。80%的NMO患者和52%的MS患者抗核抗体呈阳性。约15%抗核抗体阳性患者被诊断为风湿性自身免疫疾病。
在本研究纳入的儿童期诊断的脱髓鞘疾病患者中，抗核抗体阳性率较高，但与风湿性自身免疫疾病的关联低于成人研究中的观察结果。
