Hachulla Eric, Clerson Pierre, Launay David, Lambert Marc, Morell-Dubois Sandrine, Queyrel Viviane, Hatron Pierre-Yves
Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, Lille, France.
J Rheumatol. 2007 Dec;34(12):2423-30. Epub 2007 Nov 1.
To describe the natural history of ischemic digital ulcers (DU) in systemic sclerosis (SSc).
This single-center, retrospective, longitudinal study identified patients by demographic data, SSc history and type, Rodnan score, tobacco use, presence of autoantibodies, ongoing treatment, and DU history.
One hundred three patients were enrolled, 46 with DU history and 57 without; 2 with DU were excluded. The mean duration of followup from the first non-Raynaud SSc symptoms was 12.3 +/- 6.3 years in patients with DU history and 12.1 +/- 7.0 years in patients without. In 43% of cases, first DU occurred within 1 year following first non-Raynaud SSc symptoms, and within 5 years in 73% of cases. In a multivariate analysis, younger patients at occurrence of first non-Raynaud SSc symptoms (HR = 0.77 per each 5 years older, 95% CI 0.66-0.90) with higher Rodnan scores (HR = 1.21 per 5 points, 95% CI 1.05-1.47) experienced earlier DU occurrences, which were delayed by vasodilator therapy (HR = 0.17, 95% CI 0.09-0.32). Patients with shorter durations between first and second DU episodes, particularly with a second episode within 2 years of the first, experienced a higher yearly incidence of DU episodes (0.85 +/- 0.57 and 0.48 +/- 0.26, respectively, if less or more than 2 yrs; p = 0.04). Throughout the duration of followup, the incidence of finger amputation was 1.2% per patient-year in patients with DU history.
Patients who are young at first sign of SSc, with high Rodnan scores and without vasodilator therapy, are at high risk of developing DU. Development of DU typically occurred within 5 years of the first non-Raynaud clinical symptom of SSc in the majority of patients.
描述系统性硬化症（SSc）中缺血性指端溃疡（DU）的自然病程。
这项单中心、回顾性纵向研究通过人口统计学数据、SSc病史及类型、罗德南评分、吸烟情况、自身抗体的存在、正在进行的治疗以及DU病史来确定患者。
共纳入103例患者，46例有DU病史，57例无；排除2例有DU的患者。有DU病史患者从首次非雷诺现象的SSc症状开始的平均随访时间为12.3±6.3年，无DU病史患者为12.1±7.0年。在43%的病例中，首次DU发生在首次非雷诺现象的SSc症状出现后的1年内，73%的病例在5年内。多因素分析显示，首次出现非雷诺现象的SSc症状时年龄较小的患者（每大5岁风险比[HR]=0.77，95%可信区间[CI]0.66 - 0.90）以及罗德南评分较高的患者（每5分HR = 1.21，95% CI 1.05 - 1.47）更早出现DU，而血管扩张剂治疗可延迟DU的发生（HR = 0.17，95% CI 0.09 - 0.32）。首次和第二次DU发作间隔时间较短的患者，尤其是首次发作后2年内出现第二次发作的患者，DU发作的年发病率更高（分别为0.85±0.57和0.48±0.26，若间隔小于或大于2年；p = 0.04）。在整个随访期间，有DU病史患者的手指截肢发生率为每年1.2%。
SSc首发症状时年轻、罗德南评分高且未接受血管扩张剂治疗的患者发生DU的风险高。大多数患者DU的发生通常在SSc首次非雷诺临床症状出现后的5年内。
