托西珠单抗治疗难治性抗 EJ 阳性 ASyS 患者:病例报告。

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL
Jin Jiajia, Li Jia, Wu Wanlong, Sun Shuhui, Wang Xiaodong, Ye Shuang
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引用次数: 0

摘要

抗合成酶综合征(ASyS)是一种自身免疫性疾病,其特点是存在氨基酰-tRNA 合成酶自身抗体,并伴有肺、关节和皮肤等多个器官受累。绝大多数患者都会出现与 ASyS 相关的间质性肺病(ILD)。肺部受累的程度对患者的预后有重要影响;快速进展性间质性肺病的发生会显著增加死亡率。治疗的主要方法是泼尼松联合传统的合成改善病情抗风湿药或一些生物改善病情抗风湿药(DMARDs)。托西珠单抗(Tocilizumab,TCZ)是一种重组人源化的抗白细胞介素(IL)-6受体单克隆抗体,也被用于治疗一些与ILD相关的系统性自身免疫性风湿病。尽管美国风湿病学会(ACR)最新的《间质性肺病治疗指南》有条件地建议不要将TCZ作为特发性炎症性肌病(IIM)-ILD进展患者的治疗选择,但TCZ在ASyS患者中的治疗效果仍不明显,尤其是对于抗非Jo1抗体的难治性病例。本报告描述了一例抗EJ抗体阳性的中国ASyS患者,他们表现为典型的近端肌无力、肌酸激酶升高、非特异性间质性肺炎(NSIP)模式的ILD,同时伴有典型的皮肤受累,如技工手。患者对包括利妥昔单抗(RTX)在内的各种治疗均有耐药性,但却从 TCZ 中获益。在这个病例中,TCZ 对具有高炎症状态的致命性 ILD 急性加重显示出良好的疗效,使疾病发作相对缓解,肺功能完全恢复,长期治疗效果良好。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Tocilizumab Treatment in a Patient of Refractory Anti-EJ Positive ASyS: A Case Report.

Anti-synthetase syndrome (ASyS) is an autoimmune disease characterized by the presence of autoantibodies to aminoacyl-tRNA synthetases accompanied with various organ involvements, including the lung, joints, and skin. The ASyS-related interstitial lung disease (ILD) can be seen in the vast majority of patients. The extent of lung involvement has a significant impact on patient prognosis; the occurrence of rapid-progressive ILD could prominently increase mortality. The mainstay of treatment is prednisone in combination with conventional synthetic disease-modifying anti-rheumatic drugs or some biologic disease-modifying anti-rheumatic drugs (DMARDs). Tocilizumab (TCZ), a recombinant humanized anti-interleukin (IL)-6 receptor monoclonal antibody, has also been used to treat some systemic autoimmune rheumatic diseases associated with ILD. Although the most recent American College of Rheumatology (ACR) Guideline for the Treatment of Interstitial Lung Disease conditionally recommends against the use of TCZ as a treatment option for people with idiopathic inflammatory myopathy (IIM)-ILD progression despite initial ILD treatment, the treatment effect of TCZ in ASyS patients remains obscure, particularly for refractory cases with anti-non-Jo1 antibodies. This report describes a case of Chinese ASyS patients with anti-EJ-positive antibodies who presented with typical proximal muscle weakness, elevated creatine kinase, and ILD with non-specific interstitial pneumonia (NSIP) pattern, along with typical skin involvement such as mechanic's hand. The patients were resistant to various treatments, including rituximab (RTX), but benefited from TCZ. In this case, TCZ shows good therapeutic efficacy in a fatal acute exacerbation of ILD with a hyperinflammatory status, resulting in a relative remission of the disease flare and full preservation of lung function with a positive long-term treatment outcome.

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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
165
审稿时长
12 weeks
期刊介绍: The AFMR is committed to enhancing the training and career development of our members and to furthering its mission to facilitate the conduct of research to improve medical care. Case reports represent an important avenue for trainees (interns, residents, and fellows) and early-stage faculty to demonstrate productive, scholarly activity.
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