Su Xiaoyan, Wu Huixia, Li Hairui, Li Jinhua, Qiao Jie, Li Yueyue, Huang Xinran, Wang Liang, Zeng Shan, Zhu Lihua
Department of Rheumatology and Immunology, the First Affiliated Hospital, Jinan University, Guangzhou, 510632, People's Republic of China.
Department of Cardiology, the First Affiliated Hospital, Jinan University, Guangzhou, 510632, People's Republic of China.
Open Access Rheumatol. 2025 Aug 31;17:185-191. doi: 10.2147/OARRR.S500619. eCollection 2025.
To evaluate the efficacy of baricitinib in combination therapy for managing refractory, rapidly progressive systemic sclerosis (SSc) with severe cardiac conduction defects and interstitial lung disease (ILD).
A 48-year-old male patient with SSc complicated by significant cardiac enlargement, third-degree atrioventricular block, heart failure, progressive ILD, and partial intestinal obstruction was included in the study. Prior treatments with mycophenolate mofetil (MMF), tacrolimus, and cyclophosphamide (CTX) had shown limited efficacy. The patient subsequently received a combination regimen of glucocorticoids, intravenous immunoglobulins, CTX, and baricitinib (4 mg daily).
The patient exhibited significant clinical improvements, including a reduction in cardiac size, restoration of sinus rhythm, and resolution of heart failure symptoms. ILD and skin sclerosis showed substantial regression. Pulmonary function tests indicated significant recovery in lung capacity and diffusion capacity. Additionally, gastrointestinal symptoms such as abdominal pain and bloating were completely resolved.
This case highlights the potential of baricitinib as an adjunctive therapy for refractory SSc with multiorgan involvement. The observed improvements in cardiac conduction defects, ILD, and skin fibrosis suggest that JAK inhibitors may offer a promising therapeutic avenue for severe SSc cases resistant to conventional treatments.
评估巴瑞替尼在联合治疗难治性、快速进展性系统性硬化症（SSc）伴严重心脏传导缺陷和间质性肺疾病（ILD）中的疗效。
一名48岁男性患者，患有SSc，并发显著心脏扩大、三度房室传导阻滞、心力衰竭、进行性ILD和部分肠梗阻，被纳入该研究。先前使用霉酚酸酯（MMF）、他克莫司和环磷酰胺（CTX）治疗效果有限。该患者随后接受了糖皮质激素、静脉注射免疫球蛋白、CTX和巴瑞替尼（每日4毫克）的联合治疗方案。
患者临床症状显著改善，包括心脏大小减小、窦性心律恢复以及心力衰竭症状缓解。ILD和皮肤硬化明显消退。肺功能测试表明肺容量和弥散能力显著恢复。此外，腹痛和腹胀等胃肠道症状完全缓解。
该病例突出了巴瑞替尼作为难治性多器官受累SSc辅助治疗的潜力。观察到的心脏传导缺陷、ILD和皮肤纤维化的改善表明，JAK抑制剂可能为难治性严重SSc病例提供一种有前景的治疗途径。
