[抗合成酶综合征的治疗策略]。

Q3 Medicine
Ran Nakashima
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引用次数: 0

摘要

抗氨基酸酰 tRNA 合成酶(ARS)抗体是特发性炎症性肌病中最常见的抗体,主要与抗合成酶综合征(ASyS)有关,该综合征有六个临床特征:关节炎、肌炎、间质性肺病(ILD)、发热、雷诺现象和机械性手。尽管 ASyS 患者通常对最初的糖皮质激素(GC)治疗反应良好,但他们的病程往往是慢性、反复发作的。对于抗ARS 阳性患者,治疗目标包括抑制疾病复发和进展,同时达到最小的糖皮质激素剂量。在这方面,有人建议使用和继续使用免疫抑制剂,如降钙素抑制剂。对于难治性 ASyS 患者来说,B 细胞去势疗法有望发挥重要作用。此外,额外的抗纤维化药物可能对进展性纤维化性 ILD 患者有益。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Treatment Strategies for Anti-Synthetase Syndrome].

Anti-aminoacyl tRNA synthetase (ARS) antibodies are the most frequent in idiopathic inflammatory myopathy, notably associated with anti-synthetase syndrome (ASyS), which is characterized by six clinical features: arthritis, myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanical hands. Although patients with ASyS often respond well to initial glucocorticoid (GC) therapy, they tend to have a chronic, recurrent disease course. In anti-ARS-positive patients, the treatment goal involves suppressing disease recurrence and progression while achieving a minimal GC dose. In this regard, the administration and continuation of immunosuppressants, such as calcineurin inhibitors, have been suggested. B-cell depletion therapies are expected to be valuable in patients with refractory ASyS. Moreover, additional antifibrotic agents may be beneficial for patients with progressive fibrosing ILD.

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来源期刊
Brain and Nerve
Brain and Nerve Medicine-Neurology (clinical)
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