Stenzel Werner, Mammen Andrew L, Gallay Laure, Holzer Marie-Therese, Kleefeld Felix, Benveniste Olivier, Allenbach Yves
Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Neuropathology, Charitéplatz 1, 10117 Berlin, Germany.
National Institutes of Health, Bethesda, Maryland, USA.
Neuromuscul Disord. 2024 Dec;45:104453. doi: 10.1016/j.nmd.2024.104453. Epub 2024 Oct 4.
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.
在特发性炎性肌病中,患有抗合成酶综合征的患者呈现出独特的临床症状,伴有肌炎、间质性肺病、关节炎、皮疹和/或发热等特征性体征。骨骼肌活检的特征性形态学特征可将抗合成酶综合征与其他形式的肌炎区分开来。在此类患者的血清中可检测到通常识别氨酰tRNA合成酶蛋白家族成员之一的自身抗体,其中抗Jo1最为常见。到目前为止,抗合成酶综合征尚无国际共识定义,因此本次研讨会承担了介绍抗合成酶综合征的临床、形态学和自身抗体特征的任务。作者还通过给出管理和治疗策略来拓展目标,最后提供了抗合成酶综合征的精确分类标准。