小儿人群脱髓鞘疾病与自身免疫性风湿性疾病的关系

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: 4

摘要

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岁的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患者患有幼年皮肌炎,另1例ms患者患有系统性红斑狼疮。3例患者(13%)有一级亲属自身免疫家族史。在80%的NMO患者和52%的ms患者中发现ANA阳性,约15%的ANA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,ANA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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Associação entre doença desmielinizante e doença reumática autoimune em uma população pediátrica

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. ANA was found positive in 80% of patients with NMO and 52% of patients with MS. About 15% of ANA‐positive patients were diagnosed with rheumatologic autoimmune disieses.

Conclusion

Among patients with demyelinating diseases diagnosed in childhood included in this study there was a high frequency of ANA positivity but a lower association with rheumatologic autoimmune diseases than that observed in studies conducted in adults.

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来源期刊
CiteScore
0.82
自引率
0.00%
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0
审稿时长
6-12 weeks
期刊介绍: RBR nasceu da necessidade de se criar um órgão oficial da SBR que pudesse divulgar a produção científica dos reumatologistas brasileiros. O primeiro número foi publicado em setembro de 1957. A partir do volume 18 (1978), passou a seis números, com periodicidade atual. A RBR, em sua trajetória, tem sido objeto de constantes mudanças, sempre visando ao seu aprimoramento e revitalização, tanto em sua apresentação como em seu conteúdo.
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