Sarfaraz Sidra, Chang Janis, Kirchhof Mark G
Division of Dermatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada.
SAGE Open Med Case Rep. 2025 Jun 12;13:2050313X251343300. doi: 10.1177/2050313X251343300. eCollection 2025.
Systemic sclerosis (SSc) is a complex disease involving vasculopathy, immune dysfunction, and fibrosis, with varied clinical presentations that complicate treatment standardization. It often affects multiple organs, including the skin, lungs, gastrointestinal tract, and kidneys. We present a 52-year-old woman with a 14-year history of diffuse cutaneous SSc with severe, treatment-resistant manifestations. She had Raynaud's disease with digital ulceration and auto-amputation, telangiectasias, sclerodactyly, esophageal scleroderma, interstitial lung disease, and extensive calcinosis requiring multiple surgeries. Her disease remained poorly controlled despite treatment with nintedanib, sevelamer, colchicine, tadalafil, and prior immunosuppressants such as prednisone and mycophenolate mofetil. We initiated a trial of upadacitinib which resulted in improved vascular and cutaneous symptoms. Overall, upadacitinib provided meaningful clinical benefits despite her refractory, multisystem disease.
系统性硬化症（SSc）是一种复杂的疾病，涉及血管病变、免疫功能障碍和纤维化，临床表现多样，使治疗标准化变得复杂。它常累及多个器官，包括皮肤、肺、胃肠道和肾脏。我们报告一名52岁女性，有14年弥漫性皮肤型SSc病史，伴有严重的、难治性表现。她患有雷诺病，伴有指端溃疡和自行截肢、毛细血管扩张、指硬皮病、食管硬皮病、间质性肺病以及需要多次手术的广泛钙质沉着。尽管使用了尼达尼布、司维拉姆、秋水仙碱、他达拉非以及先前的免疫抑制剂如泼尼松和霉酚酸酯进行治疗，她的病情仍控制不佳。我们启动了一项关于乌帕替尼的试验，结果血管和皮肤症状得到改善。总体而言，尽管她患有难治性多系统疾病，但乌帕替尼仍带来了有意义的临床益处。
