Bahramian Saeed, Fazeli Patrick, Rafati Arezou, Demokri Sardar, Memari Huria, Soheili Amirali, Esmaeili Farzad, Pourmehdi Ardebili Mohammad, Erfani Haniye, Vahabi Seyed Mohammad
School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Division of Biology & Medicine, Brown University, Providence, Rhode Island, USA.
Dermatol Res Pract. 2025 Sep 12;2025:9127126. doi: 10.1155/drp/9127126. eCollection 2025.
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an autoinflammatory disease with a wide spectrum of manifestations and no standard treatment. Janus kinase inhibitors (JAK-I) are small-molecule drugs that affect many molecular pathways. We aim to investigate the safety and efficacy of JAK-I in the treatment of VEXAS syndrome. A systematic search was conducted using MeSH terms/keywords related to JAK-I and VEXAS syndrome through PubMed/Medline, Scopus, Web of Science, and Embase until July 6, 2025. We included 29 articles: 8 cohort, 8 case series, and 13 case reports. Our study includes data for 186 cases. The mean age was 69.64 years, and 83.33% were male. The most frequent manifestations were skin lesions (64.51%), fever (64.51%), arthritis and arthralgia (61.29%), lung involvement (31.72%), and venous thrombosis (24.19%). In general, 33.87% had a complete response, and 29.57% had a partial response. Ruxolitinib was used in 117 patients. Thirty-four out of 117 (29.06%) experienced complete to partial remission. Tofacitinib was used in 31 patients. About 29% of them showed complete to partial remission. Baricitinib was used in 25 patients; 12% had complete remission, and 16% had partial remission. Upadacitinib was used in 13 patients, which led to a complete remission in 38.46%. Filgotinib was used in four patients, leading to partial remission in one case. Among all, 36.55% showed adverse effects. Of these, eight were on Ruxolitinib, two on Tofacitinib, two on Baricitinib, and three on Upadacitinib. JAK-I seems to be a promising treatment option with tolerable adverse effects for VEXAS syndrome.
空泡、E1酶、X连锁、自身炎症性、体细胞（VEXAS）综合征是一种临床表现多样且无标准治疗方法的自身炎症性疾病。Janus激酶抑制剂（JAK-I）是一类影响多种分子途径的小分子药物。我们旨在研究JAK-I治疗VEXAS综合征的安全性和有效性。通过PubMed/Medline、Scopus、Web of Science和Embase，使用与JAK-I和VEXAS综合征相关的医学主题词/关键词进行系统检索，直至2025年7月6日。我们纳入了29篇文章：8篇队列研究、8篇病例系列研究和13篇病例报告。我们的研究包括186例患者的数据。平均年龄为69.64岁，83.33%为男性。最常见的表现为皮肤病变（64.51%）、发热（64.51%）、关节炎和关节痛（61.29%）、肺部受累（31.72%）和静脉血栓形成（24.19%）。总体而言，33.87%有完全缓解，29.57%有部分缓解。117例患者使用了鲁索替尼。117例中的34例（29.06%）实现了完全至部分缓解。31例患者使用了托法替布。其中约29%表现出完全至部分缓解。25例患者使用了巴瑞替尼；12%完全缓解，16%部分缓解。13例患者使用了乌帕替尼，38.46%实现了完全缓解。4例患者使用了非戈替尼，1例实现了部分缓解。其中，36.55%出现了不良反应。其中，8例使用鲁索替尼，2例使用托法替布，2例使用巴瑞替尼，3例使用乌帕替尼。JAK-I似乎是治疗VEXAS综合征的一种有前景的选择，不良反应可耐受。
