Tang Dabin, Jiang Yubao, Zhang Yanying, Xie Jingjing, Zhang Jianyong
The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, Shenzhen, Guangdong, China.
Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, Shenzhen, Guangdong, China.
Front Med (Lausanne). 2025 Jul 28;12:1591431. doi: 10.3389/fmed.2025.1591431. eCollection 2025.
Still's disease (SD) is a rare systemic autoinflammatory disorder of unknown etiology, clinically characterized by a triad of high-spiking fevers, an evanescent salmon-colored rash, and arthritis. The disease exhibits considerable heterogeneity, ranging from mild manifestations to severe, life-threatening complications such as macrophage activation syndrome (MAS). Management strategies are tailored according to disease severity, with glucocorticoids remaining the mainstay of treatment for moderate to severe cases. However, a subset of patients becomes glucocorticoid-dependent or refractory, necessitating the use of steroid-sparing immunosuppressive agents. Recent advancements in biologic therapies have significantly improved disease outcomes and are increasingly adopted as first-line therapies. The Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling pathway, known to be activated in various autoimmune and inflammatory conditions, has emerged as a promising therapeutic target. Herein, we present a case of SD complicated by severe liver injury, which was successfully managed with baricitinib following the failure of conventional synthetic disease-modifying antirheumatic drugs.
斯蒂尔病（SD）是一种病因不明的罕见全身性自身炎症性疾病，临床特征为高热、一过性鲑鱼色皮疹和关节炎三联征。该疾病表现出相当大的异质性，从轻度表现到严重的、危及生命的并发症，如巨噬细胞活化综合征（MAS）。治疗策略根据疾病严重程度进行调整，糖皮质激素仍然是中度至重度病例的主要治疗方法。然而，一部分患者会出现糖皮质激素依赖或难治的情况，因此需要使用能减少类固醇用量的免疫抑制剂。生物疗法的最新进展显著改善了疾病预后，并越来越多地被用作一线治疗方法。已知在各种自身免疫和炎症性疾病中被激活的Janus激酶-信号转导子和转录激活子（JAK-STAT）信号通路，已成为一个有前景的治疗靶点。在此，我们报告一例并发严重肝损伤的斯蒂尔病病例，在传统合成抗风湿药物治疗失败后，使用巴瑞替尼成功治疗。
