Ning Jing, Liu Qinghong, Guan Linger, He Jing
Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing, China.
Int J Rheum Dis. 2025 Apr;28(4):e70225. doi: 10.1111/1756-185X.70225.
Primary Sjögren's disease (SjD) is a systemic autoimmune disorder that primarily affects the salivary and lacrimal glands. It has been associated with immune thrombocytopenia (ITP) in about 12% of cases. Current research into new treatment strategies for patients with SjD with ITP is limited. In this report, we present the case of a 38-year-old woman who has suffered from SjD and ITP for over 20 years. Despite undergoing treatment with prednisone, mycophenolate mofetil, rituximab, tacrolimus, and cyclosporine A, she has been unable to achieve a normalization of her platelet count. Eventually, tofacitinib was introduced into her treatment plan, which led to stable platelet counts and allowed for the complete discontinuation of prednisone. She has maintained this stability for more than 2 years so far. This case underscores the potential role of tofacitinib in managing SjD-ITP, particularly for patients who do not respond adequately or are intolerant to conventional immunosuppressive therapies.
原发性干燥综合征（SjD）是一种主要累及唾液腺和泪腺的全身性自身免疫性疾病。在约12%的病例中，它与免疫性血小板减少症（ITP）相关。目前针对合并ITP的SjD患者的新治疗策略研究有限。在本报告中，我们介绍了一名38岁女性的病例，她患有SjD和ITP超过20年。尽管接受了泼尼松、霉酚酸酯、利妥昔单抗、他克莫司和环孢素A治疗，但她的血小板计数仍未能恢复正常。最终，托法替布被纳入她的治疗方案，这使血小板计数稳定，并允许完全停用泼尼松。到目前为止，她已维持这种稳定状态超过2年。该病例强调了托法替布在管理SjD-ITP中的潜在作用，特别是对于那些对传统免疫抑制疗法反应不佳或不耐受的患者。
