Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal.

IF 3.6 2区 医学 Q2 RHEUMATOLOGY Journal of Rheumatology Pub Date : 2024-11-01 DOI:10.3899/jrheum.2023-1113
Anastasios Makris, Alexandros Panagiotopoulos, Oliver Distler, Petros P Sfikakis
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Abstract

Objective: Systemic sclerosis sine scleroderma (ssSSc), formally described in 1962, is a subset of SSc that, unlike limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) forms, lacks skin fibrosis. According to the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria, SSc can be diagnosed in the absence of skin thickening, even if this is expected to develop later in disease course. Driven by a fatal case of ssSSc with cardiac involvement, we analyzed published data on ssSSc prevalence and severity.

Methods: A systematic literature review and qualitative synthesis of SSc cohorts with data on ssSSc were performed.

Results: Thirty-five studies involving a total of 25,455 patients with SSc, published between 1976 and 2023, were identified. Although different definitions were used, the mean prevalence of ssSSc was almost 10% (range 0-23%), with the largest study reporting a cross-sectional prevalence of 13%. In 5 studies with a follow-up period of up to 9 years, reclassification of ssSSc into lcSSc or dcSSc ranged 0-28%. Interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and cardiac diastolic dysfunction were present in 46% (range 9.3-59.1%), 15% (range 5.9-24.6%), 5% (range 1.6-24.6%), and 26.5% (range 1.8-40.7), respectively, of patients with ssSSc. Survival across studies was comparable to lcSSc and better than dcSSc.

Conclusion: Published data on ssSSc vary widely on prevalence, clinical expression, and prognosis, partly due to underdiagnosis and misclassification. Although classification criteria should not affect appropriate management of patients, updated ssSSc subclassification criteria that takes into account time from disease onset should be considered.

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无硬皮病的系统性硬化症:重新评估的时机。
目的:无硬皮病的系统性硬化症(ssSSc)于 1962 年被正式描述,是 SSc 的一个亚型,与局限型(lcSSc)和弥漫型皮肤病(dcSSc)不同,它没有皮肤纤维化。根据 2013 年 ACR/EULAR 标准,在没有皮肤增厚的情况下也可诊断为 SSc,即使皮肤增厚预计会在病程后期出现。在一例心脏受累的致命性 SSSC 病例的推动下,我们分析了有关 SSSC 患病率和严重程度的已发表数据:方法:我们对有 ssSSc 数据的 SSc 队列进行了系统的文献综述和定性分析:结果:共发现了 35 项研究,涉及 25,455 名 SSc 患者,这些研究发表于 1976 年至 2023 年之间。尽管采用了不同的定义,但ssSSc的平均患病率接近10%(范围为0-23%),其中最大的一项研究报告的横断面患病率为13%。在随访期长达 9 年的 5 项研究中,将 ssSSc 重新分类为 lcSSc 或 dcSSc 的比例从 0% 到 28% 不等。46%(范围 9.3-59.1%)、15%(范围 5.9-24.6%)、5%(范围 1.6-24.6%)和 26.5%(范围 1.8-40.7%)的 ssSSc 患者存在间质性肺病、肺动脉高压、硬皮病肾危象和心脏舒张功能障碍。各项研究的生存率与lcSSc相当,优于dcSSc:已发表的关于 ssSSc 的数据在患病率、临床表现和预后方面差异很大,部分原因是诊断不足和分类错误。虽然分类标准不应影响对患者的适当管理,但应考虑更新 ssSSc 亚分类标准,将发病时间考虑在内。
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来源期刊
Journal of Rheumatology
Journal of Rheumatology 医学-风湿病学
CiteScore
6.50
自引率
5.10%
发文量
285
审稿时长
1 months
期刊介绍: The Journal of Rheumatology is a monthly international serial edited by Earl D. Silverman. The Journal features research articles on clinical subjects from scientists working in rheumatology and related fields, as well as proceedings of meetings as supplements to regular issues. Highlights of our 41 years serving Rheumatology include: groundbreaking and provocative editorials such as "Inverting the Pyramid," renowned Pediatric Rheumatology, proceedings of OMERACT and the Canadian Rheumatology Association, Cochrane Musculoskeletal Reviews, and supplements on emerging therapies.
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