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青少年肌炎的扩展谱系。儿童肌炎特异性自身抗体。

A broadened spectrum of juvenile myositis. Myositis-specific autoantibodies in children.

作者信息

Rider L G, Miller F W, Targoff I N, Sherry D D, Samayoa E, Lindahl M, Wener M H, Pachman L M, Plotz P H

机构信息

Food and Drug Administration, Bethesda, Maryland 20892.

出版信息

Arthritis Rheum. 1994 Oct;37(10):1534-8. doi: 10.1002/art.1780371019.

Abstract

OBJECTIVE

Myositis-specific autoantibodies (MSA) define relatively homogeneous clinical and immunogenetic patient groups in adults with idiopathic inflammatory myopathies (IIM). This study explores the usefulness of MSA in defining groups of children with myositis.

METHODS

Sera from 77 children with myositis and other connective tissue diseases were tested for MSA by immunoprecipitation and immunodiffusion. Clinical data were collected and analyzed.

RESULTS

The MSA anti-PL-12 (alanyl-transfer RNA synthetase), anti-Jo-1 (histidyl-tRNA synthetase), anti-signal recognition particle, and anti-Mi-2 were each identified in the sera of 12 children with IIM. In these patients, the clinical manifestations, disease courses, and responses to therapy closely resembled those in adults with the same autoantibodies.

CONCLUSION

These observations suggest that the clinical syndromes defined by particular MSA are similar in children and adults with IIM. By defining similar clinical syndromes in children who have MSA, this study provides a basis for future studies of MSA in the idiopathic inflammatory myopathies of childhood, which may be useful in predicting the clinical courses of a subset of these patients and improving their therapy.

摘要

目的

在患有特发性炎性肌病(IIM)的成人中,肌炎特异性自身抗体(MSA)可界定相对同质化的临床和免疫遗传学患者群体。本研究探讨MSA在界定儿童肌炎患者群体方面的效用。

方法

采用免疫沉淀和免疫扩散法对77例患有肌炎及其他结缔组织疾病的儿童血清进行MSA检测。收集并分析临床数据。

结果

在12例IIM患儿血清中分别鉴定出MSA抗PL-12(丙氨酰 - 转运RNA合成酶)、抗Jo-1(组氨酰 - tRNA合成酶)、抗信号识别颗粒和抗Mi-2。在这些患儿中,临床表现、病程及对治疗的反应与具有相同自身抗体的成人极为相似。

结论

这些观察结果表明,在患有IIM的儿童和成人中,由特定MSA所界定的临床综合征相似。通过在患有MSA的儿童中界定相似的临床综合征,本研究为未来关于儿童特发性炎性肌病中MSA的研究奠定了基础,这可能有助于预测部分此类患者的临床病程并改善其治疗。

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