E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy.
J Rheumatol. 2021 Jan 1;48(1):82-86. doi: 10.3899/jrheum.190976. Epub 2020 Mar 15.
To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.
We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.
One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.
This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.
为了验证这样一个假设，即早期系统性硬化症（SSc）患者是一个异质性群体，具有轻度或早期疾病，我们分析了这些患者在临床、流行病学和免疫学特征方面的异质性程度。
我们对苏黎世队列中的早期 SSc 患者进行了分析，这些患者既不符合 2013 年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）标准，也不符合 1980 年 ACR 分类标准，但具有临床专家诊断的 SSc 伴有雷诺现象（RP）和 SSc 的其他特征（肿胀的手指、SSc 特异性抗体、甲襞毛细血管镜上的 SSc 模式或任何特征性的 SSc 器官受累）。疾病持续时间从首次 RP 症状开始定义。
102 名符合纳入标准的患者被纳入分析。他们的临床表现具有异质性，绝大多数患者表现为 RP、抗核抗体和甲襞毛细血管镜改变，但也有其他特征的不同表现，如 SSc 特异性抗体和早期器官受累迹象。虽然 54.1%（52/96）的患者疾病持续时间<5 年，但多达 29.1%（28/96）的患者疾病持续时间>10 年，表明存在长期轻度疾病。在首次就诊时的临床特征方面，早期、潜在进展性疾病患者与长期轻度疾病患者没有差异。
这项研究表明，早期 SSc 患者是一个具有轻度或早期疾病的混合群体。这在临床实践中需要考虑到风险分层和考虑为早期 SSc 的患者的研究设计。
