系统性硬皮病-系统性红斑狼疮重叠综合征的流行病学和生存情况。
Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome.
机构信息
From the Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Division of Rheumatology, Mount Sinai Hospital, University of Toronto; Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto, Toronto, Ontario, Canada; Taibah University, Medina, Saudi Arabia.
S. Alharbi, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto, and Taibah University; Z. Ahmad, MD, Division of Rheumatology, Mount Sinai Hospital, University of Toronto; A.A. Bookman, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Z. Touma, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto; J. Sanchez-Guerrero, MD, MSc, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto; N. Mitsakakis, PhD, Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, University of Toronto, and Division of Rheumatology, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto.
出版信息
J Rheumatol. 2018 Oct;45(10):1406-1410. doi: 10.3899/jrheum.170953. Epub 2018 Jul 15.
OBJECTIVE
Systemic sclerosis (SSc) may overlap with systemic lupus erythematous (SLE). Little is known about the epidemiology, clinical characteristics, and survival of SSc-SLE overlap. We evaluated the prevalence of SSc-SLE overlap and differences in SSc characteristics, and compared survival with SSc without SLE.
METHODS
A cohort study was conducted including subjects who fulfilled the American College of Rheumatology (ACR)/European League Against Rheumatism classification criteria for SSc and/or the ACR criteria for SLE. The primary outcome was time from diagnosis to all-cause mortality. Survival was evaluated using Kaplan-Meier and Cox proportional hazard models.
RESULTS
We identified 1252 subjects (SSc: n = 1166, SSc-SLE: n = 86) with an SSc-SLE prevalence of 6.8%. Those with SSc-SLE were younger at diagnosis (37.9 yrs vs 47.9 yrs, p < 0.001), more frequently East Asian (5.5% vs 20%) or South Asian (5.1% vs 12%), had lupus anticoagulant (6% vs 0.3%, p < 0.001), anticardiolipin antibody (6% vs 0.9%, p < 0.001), and pulmonary arterial hypertension (PAH; 52% vs 31%, p < 0.001). Those with SSc-SLE less frequently had calcinosis (13% vs 27%, p = 0.007), telangiectasia (49% vs 75%, p < 0.001), and diffuse subtype (12% vs 35%, p < 0.001). There were no significant differences in the occurrence of renal crisis (7% vs 7%), interstitial lung disease (ILD; 41% vs 34%), and digital ulcers (38% vs 32%). Those with SSc-SLE had better median survival time (26.1 vs 22.4 yrs), but this was not statistically significant (log-rank p = 0.06). Female sex and diffuse subtype attenuated survival differences between groups (HR 1.07, 95% CI 0.67-1.67).
CONCLUSION
Patients with SSc-SLE are younger at diagnosis, more frequently have PAH, and less frequently have cutaneous manifestations of SSc. They should be monitored for ILD, renal crisis, and digital ulcers.
目的
系统性硬化症（SSc）可能与系统性红斑狼疮（SLE）重叠。关于 SSc-SLE 重叠的流行病学、临床特征和生存情况知之甚少。我们评估了 SSc-SLE 重叠的患病率以及 SSc 特征的差异，并与无 SLE 的 SSc 进行了生存比较。
方法
进行了一项队列研究，纳入了符合美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）SSc 分类标准和/或 ACR SLE 标准的患者。主要结局为从诊断到全因死亡率的时间。使用 Kaplan-Meier 和 Cox 比例风险模型评估生存情况。
结果
我们确定了 1252 名患者（SSc：n = 1166，SSc-SLE：n = 86），SSc-SLE 的患病率为 6.8%。SSc-SLE 患者的诊断年龄更小（37.9 岁 vs 47.9 岁，p < 0.001），东亚裔（5.5% vs 20%）或南亚裔（5.1% vs 12%）的比例更高，存在狼疮抗凝物（6% vs 0.3%，p < 0.001）、抗心磷脂抗体（6% vs 0.9%，p < 0.001）和肺动脉高压（PAH；52% vs 31%，p < 0.001）的比例更高。SSc-SLE 患者发生钙化（13% vs 27%，p = 0.007）、毛细血管扩张（49% vs 75%，p < 0.001）和弥漫性亚型（12% vs 35%，p < 0.001）的比例更低。肾危象（7% vs 7%）、间质性肺病（ILD；41% vs 34%）和指端溃疡（38% vs 32%）的发生率无显著差异。SSc-SLE 患者的中位生存时间更长（26.1 年 vs 22.4 年），但差异无统计学意义（对数秩检验 p = 0.06）。女性和弥漫性亚型降低了两组之间的生存差异（HR 1.07，95%CI 0.67-1.67）。
结论
SSc-SLE 患者的诊断年龄更小，更常发生 PAH，皮肤 SSc 表现更少。应监测 ILD、肾危象和指端溃疡。
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