Department of Rheumatology, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Rheumatology (Oxford). 2020 Oct 1;59(10):2920-2929. doi: 10.1093/rheumatology/keaa026.
SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc.
A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012. First we identified every case in the country registered with an International Classification of Diseases, Tenth Revision code for SSc (M34). Next we manually reviewed all cases coded as M34 to determine whether they met the 1980 ACR and/or 2013 ACR/EULAR classification criteria for SSc and could be included in the Norwegian SSc cohort (Nor-SSc). Finally, all disease features from SSc onset to study end were reviewed.
The Nor-SSc cohort included 815 SSc patients. The mean age at diagnosis was 53 years, with 84% females and 77% limited cutaneous SSc. The estimated incidence increased from 4 per million in 2000 to 13 per million in 2012. We identified high cumulative frequencies of internal organ involvement, coexistence of multiple organ afflictions across disease subsets and autoantibody status and stable frequencies of pulmonary arterial hypertension across haemodynamic definitions, but indications of referral-related differences in pulmonary hypertension detection rates across the study area.
This nationwide cohort study provides new, unbiased evidence for a high disease burden in SSc patients of Caucasian descent and indicates the existence of hurdles preventing equality of assessment across the SSc population.
SSc 是一种严重的、异质性的多器官疾病，目前缺乏基于人群的表型谱、总体疾病负担和社会影响的估计。本研究旨在提供 SSc 患者表型和主要器官受累的首个全国性数据。
采用逐步策略在挪威寻找并描述 2000 年至 2012 年期间的每一位 SSc 患者。首先，我们在全国范围内确定了每一个登记有 SSc（M34）国际疾病分类第十版代码的病例。然后，我们手动审查了所有编码为 M34 的病例，以确定它们是否符合 1980 年 ACR 和/或 2013 年 ACR/EULAR 的 SSc 分类标准，并可以纳入挪威 SSc 队列（Nor-SSc）。最后，我们回顾了从 SSc 发病到研究结束的所有疾病特征。
Nor-SSc 队列包括 815 名 SSc 患者。诊断时的平均年龄为 53 岁，女性占 84%，局限性皮肤 SSc 占 77%。估计发病率从 2000 年的每百万 4 例增加到 2012 年的每百万 13 例。我们发现，内脏器官受累的累积频率高，不同疾病亚组之间存在多种器官受累共存和自身抗体状态，肺动脉高压的频率稳定，不同血流动力学定义之间存在差异，但在研究区域内提示肺动脉高压检出率存在转诊相关差异。
这项全国性队列研究提供了白种人 SSc 患者高疾病负担的新的、无偏倚的证据，并表明存在阻碍 SSc 人群评估平等的障碍。
