273rd ENMC International workshop: Clinico-Sero-morphological classification of the Antisynthetase syndrome. Amsterdam, The Netherlands, 27-29 October 2023

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2024-10-04 DOI:10.1016/j.nmd.2024.104453

Werner Stenzel , Andrew L Mammen , Laure Gallay , Marie-Therese Holzer , Felix Kleefeld , Olivier Benveniste , Yves Allenbach

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引用次数: 0

Abstract

Among the idiopathic inflammatory myopathies, patients harbouring an Antisynthetase syndrome exhibit a unique clinical picture, with characteristic signs such as myositis, interstitial lung disease, arthritis, rash, and/or fever. Characteristic morphological features on skeletal muscle biopsies differentiate Antisynthetase syndrome from other forms of myositis. Autoantibodies typically recognizing one of the members of the aminoacyl-tRNA synthetase family of proteins can be detected in the serum of such patients, with anti-Jo1 being most frequent. Until now, an international consensus definition of the Antisynthetase syndrome is lacking, hence this workshop has undertaken the task to inform about the clinical, morphological and autoantibody profiles of Antisynthetase syndrome. The authors also expand their aims by giving management and therapeutic strategies, and finally provide precise classification criteria for Antisynthetase syndrome.

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第 273 届 ENMC 国际研讨会：抗合成酶综合征的临床-零形态学分类。荷兰阿姆斯特丹，2023 年 10 月 27-29 日

在特发性炎症性肌病中，Antisynthetase 综合征患者表现出独特的临床症状，如肌炎、间质性肺病、关节炎、皮疹和/或发热。骨骼肌活检的特征性形态学特征可将抗纹状体酶综合征与其他形式的肌炎区分开来。在这类患者的血清中可检测到典型的识别氨基酰-tRNA合成酶家族蛋白之一的自身抗体，其中以抗Jo1最为常见。迄今为止，国际上尚未就抗淀粉酶综合征的定义达成共识，因此本次研讨会的任务是介绍抗淀粉酶综合征的临床、形态和自身抗体特征。作者们还通过提供管理和治疗策略扩展了他们的目标，并最终为抗胰蛋白酶综合征提供了精确的分类标准。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.