系统性硬化症患者出现指趾端溃疡提示预后不佳

摘要 目的：系统性硬化症（systemic sclerosis，SSc）是一种致残率、死亡率均较高的系统性自身免疫病。学界对能够评测系统性硬化症患者危险分层的临床因素、早期治疗以确保最佳临床效果的呼声甚高。在本研究中，评估就诊时既往指趾端溃疡是否为系统性硬化症患者心血管方面预后及整体临床恶化及死亡的预测因素之一。

方法：患者来自EULAR硬皮病试验研究数据库，均满足美国风湿病学会1980年版系统性硬化症分类标准。入选本研究的患者包括随访至少3年的和已经死亡的患者。应用COX比例风险回归分析，分析就诊时指趾端溃疡史是否可以作为临床恶化及死亡的预测因素之一。

结果：符合入选标准的有3196例患者（男性13.2%，占数据库的33.4%）。就诊时，3196例患者中的1092例有指趾端溃疡史（占34.1%）。在校正年龄、性别及其他具有潜在显著性参数等因素后，多因素分析显示指趾端溃疡史可预测随后随访中出现活动性指趾端溃疡（HR2.41，95%CI 1.91～3.03，P＜0.001）、心脏超声示肺动脉高压（HR1.36，95%CI 1.03～1.80，P=0.032）、心血管事件（新发指趾端溃疡、肺动脉高压、左室功能不全）（HR 3.56，95%CI 2.26～5.62，P＜0.001）及死亡（HR 1.53，95%CI 1.16～2.02，P=0.003）（表5）。

表 指趾端溃疡史对系统性硬化症患者临床恶化及死亡的预测性

?经校正年龄、性别及其他具有潜在显著性参数等多因素，?肺动脉收缩压＞40mmHg

结论：系统性硬化症患者指趾端溃疡既往史可预测随后随访中出现活动性指趾端溃疡，并与心血管临床恶化及存活率降低相关。

附原文:Abstract OBJECTIVE:Systemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc.METHODS:Patients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3?years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis.RESULTS:3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), p<0 .001, for an elevated systolic pulmonary arterial pressure on heart ultrasound (us-paps):1.36 (1.03 to 1.80), p="0.032," for any cardiovascular event (new dus, elevated us-paps or lv failure): 3.56 (2.26 to 5.62), p<0.001, and for death (1.53 (1.16 to 2.02), p="0.003).CONCLUSIONS:In" patients with ssc, hdu at presentation predicts the occurrence of dus at follow-up and is associated with cardiovascular worsening and decreased survival.

引自: Mihai C, Landewé R, van der Heijde D, et al.Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis， 2016，75(4):681-6.