Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
Amsterdam Rheumatology Center/University of Amsterdam & Atrium Medical Center, Heerlen, The Netherlands.
Ann Rheum Dis. 2016 Apr;75(4):681-6. doi: 10.1136/annrheumdis-2014-205897. Epub 2015 Feb 16.
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.
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.
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).
In patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival.
系统性硬化症（SSc）是一种系统性自身免疫性疾病，发病率高，死亡率高。非常需要预测因子，以便对 SSc 患者进行风险分层，最终尽早开始治疗，以确保最佳的临床结果。在这项研究中，我们评估了初诊时是否存在手指溃疡（HDU）病史是否可以预测 SSc 患者的血管结局以及整体临床恶化和死亡。
纳入符合 1980 年美国风湿病学会分类标准的 SSc 患者，且至少有 3 年的基线随访或已死亡的患者，EULAR 硬皮病试验和研究（EUSTAR）数据库中的患者被纳入分析。通过 Cox 比例风险回归分析评估初诊时的 HDU 是否为疾病恶化或死亡的预测因素。
3196 名患者符合纳入标准（男性占 13.2%，弥漫性亚组占 33.4%）。初诊时，3196 名患者中有 1092 名（34.1%）存在 HDU。在多变量分析中，调整了年龄、性别和所有被认为有潜在意义的参数后，HDU 可预测前瞻性就诊时存在活动性手指溃疡（DUs）（HR（95%CI））：2.41（1.91 至 3.03），p<0.001），超声心动图（US-PAPs）显示收缩期肺动脉压升高（HR（95%CI））：1.36（1.03 至 1.80），p=0.032），任何心血管事件（新发 DUs、US-PAPs 升高或 LV 衰竭）（HR（95%CI））：3.56（2.26 至 5.62），p<0.001），以及死亡（HR（95%CI））：1.53（1.16 至 2.02），p=0.003）。
在 SSc 患者中，初诊时的 HDU 可预测随访时发生 DUs，并且与心血管恶化和生存率降低相关。
