Dermatology Hospital of Jiangxi Province, Jiangxi Provincial Clinical Research Center for Skin Diseases, Candidate Branch of National Clinical Research Center for Skin Diseases, Nanchang, 330000, China.
Jiangxi Medical College of Nanchang University, Nanchang, 330000, China.
Drugs R D. 2024 Sep;24(3):391-394. doi: 10.1007/s40268-024-00487-1. Epub 2024 Sep 18.
Adult-onset Still's disease (AOSD) is a recognized autoinflammatory disorder of unknown etiology. The standard initial management for AOSD includes conventional corticosteroids and disease-modifying antirheumatic drugs. In cases that are resistant to these treatments, additional therapeutic options such as immunosuppressants, biologics, and other alternative treatments may be considered. Yet, a significant proportion of patients remain unresponsive to these therapeutic interventions. Herein, a case is reported involving a patient with AOSD who had persistent pruritic lesions that did not respond to conventional therapy, but were alleviated with Janus kinase inhibitors (JAKi), namely baricitinib and upadacitinib. The objective is to expand the number of refractory AOSD cases treated with JAKi in clinical practice. Another aim is to offer potentially effective therapeutic options for AOSD patients who experience persistent pruritus.
A case was reported involving AOSD characterized by persistent pruritic lesions that failed to respond to conventional treatment, but showed favorable outcomes with JAKi therapy. An analysis of the PubMed literature was performed to assess the medication's efficacy and explore possible mechanisms.
The present case study is one of the few documented instances exploring the use of JAKi for treating AOSD, aligning with previously published research. After initiating JAKi therapy, the patient exhibited significant improvement in symptoms, most notably a reduction in persistent pruritus. Additionally, there was a substantial decrease in the patient's glucocorticoid dosage. Aside from minor renal function anomalies, no adverse reactions were observed.
The present case illustrates that JAKi can provide rapid and sustained clinical improvement in patients with AOSD, especially those who have not responded to conventional treatment, and they have the ability to alleviate persistent itching. Further investigation is needed to ascertain the precise mechanism.
成人Still 病（AOSD）是一种病因不明的公认的自身炎症性疾病。AOSD 的标准初始治疗包括常规皮质类固醇和改善病情的抗风湿药物。在这些治疗方法无效的情况下，可能会考虑其他治疗选择，如免疫抑制剂、生物制剂和其他替代治疗。然而，仍有相当一部分患者对这些治疗干预无反应。在此，报告了一例 AOSD 患者，其持续性瘙痒性皮损对常规治疗无反应，但用 Janus 激酶抑制剂（JAKi），即巴瑞替尼和乌帕替尼治疗后得到缓解。目的是在临床实践中增加用 JAKi 治疗难治性 AOSD 的病例数量。另一个目的是为经历持续性瘙痒的 AOSD 患者提供潜在有效的治疗选择。
报告了一例 AOSD 病例，其特征为持续性瘙痒性皮损对常规治疗无效，但 JAKi 治疗后结果良好。对 PubMed 文献进行了分析，以评估药物的疗效并探讨可能的机制。
本病例研究是为数不多的探索 JAKi 治疗 AOSD 的病例之一，与先前发表的研究一致。开始 JAKi 治疗后，患者的症状显著改善，特别是持续性瘙痒明显减轻。此外，患者的糖皮质激素剂量也大幅减少。除了轻微的肾功能异常外，未观察到不良反应。
本病例表明，JAKi 可迅速和持续改善 AOSD 患者的临床症状，尤其是那些对常规治疗无反应的患者，且有能力缓解持续性瘙痒。需要进一步研究以确定确切的机制。
