Nora Hajnoczky, L Scott Levin, Nora Sandorfi, Chris T Derk
{"title":"Long-term outcomes of vascular grafting of medium-sized vessels of wrist and hand in systemic sclerosis patients with medication refractory Raynaud's.","authors":"Nora Hajnoczky, L Scott Levin, Nora Sandorfi, Chris T Derk","doi":"10.1177/23971983251332044","DOIUrl":null,"url":null,"abstract":"<p><p><b>Objective:</b> 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. <b>Methods:</b> 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. <b>Results:</b> 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. <b>Conclusion:</b> The retrospective study assessed the long-term benefits of arterial revascularization in medication refractory systemic sclerosis-Raynaud's patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251332044"},"PeriodicalIF":1.4000,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12018359/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Scleroderma and Related Disorders","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/23971983251332044","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Objective: 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. Methods: 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. Results: 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. Conclusion: The retrospective study assessed the long-term benefits of arterial revascularization in medication refractory systemic sclerosis-Raynaud's patients.