Hajnoczky Nora, Levin L Scott, Sandorfi Nora, Derk Chris T
Einstein Healthcare Network, Philadelphia, PA, USA.
Department of Orthopaedic Surgery, Penn Nerve Center, University of Pennsylvania, Philadelphia, PA, USA.
J Scleroderma Relat Disord. 2025 Apr 22:23971983251332044. doi: 10.1177/23971983251332044.
Systemic sclerosis is characterized by vasculopathy at the microvascular level and less commonly the macrovascular level. The vasculopathy in systemic sclerosis, based on its functional and structural changes, leads to symptoms of vasospasm (Raynaud's phenomenon) and tissue injury. In most cases, preventive actions and pharmacotherapy provide beneficial treatment options; however, in severe cases, painful ulcerations, gangrene, and digital amputations may occur with treatment. In medication refractory cases, revascularization of medium and small vessels has been pursued. Other studies regarding long-term outcomes of revascularization of systemic sclerosis-Raynaud's patients are less reported. Patients at the University of Pennsylvania (2009-2022) were identified with International Classification of Diseases (ICD)-9 and ICD-10 diagnosis codes for systemic sclerosis (ACR/EULAR 2013 criteria), Raynaud's phenomenon, and vascular grafting. Retrospective assessment of preoperative and postoperative patient-reported symptoms (Raynaud's symptoms, pain, ulcers), exam findings, and imaging results were collected. Twelve hands from 10 patients were identified. Nine patients had limited systemic sclerosis, and one patient had diffuse systemic sclerosis. Eight patients were female, and the average surgery age was 52.3 ± 17.7 years. Vessel occlusion and revascularization occurred in ulnar artery of 11 hands and radial artery of 1 hand. Patients were followed up for an average of 45.4 ± 38.7 months, with three patient deaths within 2 years. Unremitting digital ulcers, pain, and color change were noted in three patients, likely due to medication mal-adherence and graft occlusion. Most hands (9/12) had subjective and objective resolution, or improvement in digital ulcers, pain, and color changes. The retrospective study assessed the long-term benefits of arterial revascularization in medication refractory systemic sclerosis-Raynaud's patients.
系统性硬化症的特征是微血管水平存在血管病变,大血管水平出现病变的情况较少见。基于其功能和结构变化,系统性硬化症中的血管病变会导致血管痉挛症状(雷诺现象)和组织损伤。在大多数情况下,预防措施和药物治疗提供了有益的治疗选择;然而,在严重病例中,治疗过程中可能会出现疼痛性溃疡、坏疽和手指截肢。对于药物难治性病例,已开展中小血管的血运重建。关于系统性硬化症 - 雷诺病患者血运重建长期结果的其他研究报道较少。宾夕法尼亚大学(2009 - 2022年)的患者通过国际疾病分类(ICD)-9和ICD - 10诊断代码确定患有系统性硬化症(ACR/EULAR 2013标准)、雷诺现象和血管移植。收集术前和术后患者报告的症状(雷诺症状、疼痛、溃疡)、检查结果和影像学结果的回顾性评估资料。确定了来自10名患者的12只手。9名患者患有局限性系统性硬化症,1名患者患有弥漫性系统性硬化症。8名患者为女性,平均手术年龄为52.3±17.7岁。11只手的尺动脉和1只手的桡动脉发生血管闭塞和血运重建。患者平均随访45.4±38.7个月,2年内有3例患者死亡。3例患者出现持续的手指溃疡、疼痛和颜色改变,可能是由于药物依从性差和移植物闭塞。大多数手(9/12)在主观和客观上手指溃疡、疼痛和颜色改变得到缓解或改善。这项回顾性研究评估了药物难治性系统性硬化症 - 雷诺病患者动脉血运重建的长期益处。