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JAK 抑制剂治疗成人幼年特发性关节炎:一项回顾性单中心经验。

JAK inhibitors in the treatment of adult patients with juvenile idiopathic arthritis: a retrospective monocentric experience.

机构信息

Department of Clinical Sciences and Community Health, University of Milan, and Paediatric Rheumatology Unit, ASST Pini-CTO, Milan, Italy.

Department of Clinical Sciences and Community Health, University of Milan; Paediatric Rheumatology Unit, and Clinical Rheumatology Unit, ASST Pini-CTO, Milan, Italy.

出版信息

Clin Exp Rheumatol. 2024 May;42(5):974-982. doi: 10.55563/clinexprheumatol/4yoas8. Epub 2024 Mar 26.

Abstract

OBJECTIVES

This study aims to evaluate the efficacy and safety of JAK inhibitors (JAKi) in a monocentric cohort of adult patients with juvenile idiopathic arthritis (JIA).

METHODS

Patients attending a rheumatology transition clinic were retrospectively included in case of: i) JIA diagnosis according to current classification criteria (1); ii) age ≥18 years and iii) treatment with JAKi for at least 3 months.

RESULTS

Seventeen adult patients with JIA were treated with JAKi (as first JAKi, 9 patients (52.9%) received tofacitinib and 8 (47.1%) baricitinib). At 3 months after JAKi initiation, 8 patients (47%) achieved a response and 4 patients (23.5%) achieved disease remission (3 patients with baricitinib and 1 with tofacitinib, 37.5% vs. 16.7%, p=0.294). None of those with systemic JIA and enthesitis-related arthritis obtained remission; the remission rate at 3 months was higher, although not significantly, in the oligoarticular subset compared to the polyarticular subset (37.5% vs. 20%). Patients with ≤1 active joint involvement at JAKi start had a higher remission rate (50% vs. 22.2%). Subjects who achieved remission on JAKi had a significantly lower pre-treatment DAS28-CRP compared to those with still active disease (p=0.010, Mann-Whitney U=4). A pre-treatment DAS28-CRP <3.76 predicted response to JAKi with 100% sensitivity and 84.6% specificity (p=0.023). The remission rate was lower among patients who had been treated with ≥2 biological drugs before JAKi start (9% vs. 66.7%; p=0.05). One patient in concomitant treatment with leflunomide developed severe arterial hypertension.

CONCLUSIONS

JAKi may represent an effective and safe treatment option for adult JIA patients with low/moderate disease activity, particularly in case of oligoarticular involvement.

摘要

目的

本研究旨在评估 JAK 抑制剂(JAKi)在单中心成人幼年特发性关节炎(JIA)患者队列中的疗效和安全性。

方法

回顾性纳入在风湿科转科门诊就诊的符合以下条件的患者:i)根据当前分类标准(1)诊断为 JIA;ii)年龄≥18 岁;iii)接受 JAKi 治疗至少 3 个月。

结果

17 例 JIA 成年患者接受 JAKi 治疗(作为一线 JAKi,9 例(52.9%)接受托法替布,8 例(47.1%)接受巴瑞替尼)。JAKi 起始后 3 个月,8 例(47%)患者达到缓解,4 例(23.5%)患者达到疾病缓解(3 例接受巴瑞替尼,1 例接受托法替布,37.5%比 16.7%,p=0.294)。无系统性 JIA 和附着点炎相关关节炎患者达到缓解;寡关节炎亚组的缓解率虽无统计学差异,但高于多关节炎亚组(37.5%比 20%)。JAKi 起始时≤1 个活动关节的患者缓解率更高(50%比 22.2%)。在 JAKi 治疗后达到缓解的患者,其治疗前 DAS28-CRP 明显低于仍有疾病活动的患者(p=0.010,Mann-Whitney U=4)。治疗前 DAS28-CRP<3.76 预测 JAKi 治疗的反应,敏感度为 100%,特异度为 84.6%(p=0.023)。JAKi 起始前接受≥2 种生物制剂治疗的患者缓解率较低(9%比 66.7%;p=0.05)。1 例同时接受来氟米特治疗的患者出现严重动脉高血压。

结论

JAKi 可能是一种有效且安全的治疗选择,适用于疾病活动度低/中度的成年 JIA 患者,尤其是寡关节炎患者。

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