The positive effect of JAK inhibitor tofacitinib in the treatment of primary Sjögren’s syndrome: a clinical case

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Abstract

Primary Sjögren’s syndrome (pSS, Sjögren’s disease) is a systemic autoimmune disease which develops in previously healthy individuals and characterized by damage to exocrine glands, mainly lacrimal and salivary glands, with gradual formation of their secretory insufficiency and various systemic manifestations. According to EULAR recommendation (2019) therapeutic management of pSS bases on symptomatic treatment of sicca syndrome and broad-spectrum immunosuppression for systemic manifestations. The perspective group for the treatment of autoimmune disease is Janus kinase inhibitors, which can block the signals from biologically active molecules (interferons, erythropoietins and cytokines) and providing a response to these target cell signals. Therefore, the use of JAK inhibitors in patients with pSS requires clinical confirmation of effectiveness. The article described the clinical case of the positive effect of tofacitinib with methotrexate combination in the treatment of patient with pSS. A 55-year-old female with sicca syndrome (confirmed by Schirmer’s test), arthralgia, low-grade fever, weight loss and a positive test for the detection of specific antibodies (SS-A/Ro > 240 units/ml, SS-B/La 94 units/ml) was diagnosed with pSS. The total score of EULAR Sjögren’s syndrome disease activity index (ESSDAI) was 9 (activity grade II). The treatment included symptomatic methods of sicca syndrome correcting (replacement therapy with artificial tear preparations and chewing gums with xylitol) in combination with immunosuppressive therapy. The addition of tofacitinib 5 mg twice daily to methotrexate (10 mg weekly) has been shown to significantly reduce disease activity after 6 months of treatment (ESSDAI = 0).
JAK抑制剂托法替尼治疗原发性Sjögren综合征1例临床观察
原发性Sjögren’s综合征(pSS, Sjögren’s disease)是一种全身性自身免疫性疾病,发生在原本健康的个体中,以外分泌腺(主要是泪腺和唾液腺)损伤为特征,逐渐形成分泌功能不全和各种全身表现。根据EULAR建议(2019),pSS的治疗管理以干枯综合征的对症治疗和广谱免疫抑制为基础。治疗自身免疫性疾病的前景组是Janus激酶抑制剂,它可以阻断来自生物活性分子(干扰素、促红细胞生成素和细胞因子)的信号,并对这些靶细胞信号作出反应。因此,在pSS患者中使用JAK抑制剂需要临床证实其有效性。本文描述了托法替尼联合甲氨蝶呤治疗pSS患者的临床病例。患者为55岁女性,伴有风疹综合征(经Schirmer试验证实)、关节痛、低烧、体重减轻,特异性抗体检测阳性(SS-A/Ro > 240单位/ml, SS-B/La 94单位/ml),诊断为pSS。EULAR Sjögren综合征疾病活跃性指数(ESSDAI)总分为9分(活跃性二级)。治疗方法包括对症治疗(人工泪制剂替代治疗和木糖醇口香糖替代治疗)联合免疫抑制治疗。在甲氨蝶呤(每周10毫克)的基础上添加tofacitinib 5毫克,每日2次,已被证明在治疗6个月后显著降低疾病活动性(ESSDAI = 0)。
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