Association between demyelinating disease and autoimmune rheumatic disease in a pediatric population

Ana Luiza M. Amorim , Nadia C. Cabral , Fabiane M. Osaku , Claudio A. Len , Enedina M.L. Oliveira , Maria Teresa Terreri
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引用次数: 2

Abstract

Introduction

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.

Objective

To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients.

Method

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.

Results

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.

Conclusion

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.

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脱髓鞘疾病和自身免疫性风湿病在儿科人群中的相关性
多发性硬化症(MS)和视神经脊髓炎(NMO)是中枢神经系统脱髓鞘疾病。脱髓鞘病患者及其家族的自身免疫已被广泛调查和讨论。最近的研究表明风湿性自身免疫性疾病在成年MS或NMO患者及其家庭中的发病率较高,但在儿科人群中没有研究。目的探讨多发性硬化症和NMO与儿童自身免疫性风湿病的关系。方法对22例年龄小于21岁、18岁前诊断为MS或NMO的患者进行流行病学资料、临床表现、与自身免疫性疾病的相关性、自身免疫性疾病家族史、实验室检查、影像学检查和自身抗体的存在进行评估。结果本组患者中,女性患病率为68.1%。出现症状的平均年龄为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病史:1例NMO患者为幼年皮肌炎,另1例ms患者为系统性红斑狼疮。3例患者(13%)有一级亲属自身免疫家族史。抗核抗体在80%的NMO患者和52%的ms患者中呈阳性,约15%的抗核抗体阳性患者被诊断为风湿病自身免疫性疾病。结论在本研究中诊断为儿童期脱髓鞘疾病的患者中,抗核抗体阳性的频率较高,但与成人研究中观察到的风湿性自身免疫性疾病的相关性较低。
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