Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
Medicine & Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
RMD Open. 2023 Apr;9(2). doi: 10.1136/rmdopen-2022-002971.
Since 2009, Dutch patients with a confirmed diagnosis/suspicion of systemic sclerosis (SSc) can be referred to the Leiden Combined Care in Systemic Sclerosis (CCISS) cohort. This study evaluated whether early recognition of SSc has improved over time and whether disease characteristics and survival has changed over time.
643 SSc patients fulfilling American College of Rheumatology/European Alliance of Associations for Rheumatology 2013 SSc criteria were included and categorised into three groups based on cohort-entry year: (1) 2010-2013 (n=229 (36%)), (2) 2014-2017 (n=207 (32%)) and (3) 2018-2021 (n=207 (32%)). Variables including disease duration, interstitial lung disease (ILD), digital ulcers (DU), diffuse cutaneous SSc (dcSSc), antitopoisomerase (ATA) and anticentromere (ACA) antibodies, and survival from disease onset were compared between cohort-entry groups, including analyses stratified for sex and autoantibodies.
Over time, duration between onset of disease symptoms and cohort entry decreased in males and females, but was always longer in females than in males.The proportion of patients presenting with DU decreased, especially in ACA+SSc patients. Almost no ACA+ patients presented with ILD, while in ATA+ patients this proportion was 25% in 2010-2013 and decreased to 19% in 2018-2021. A reduction in patients presenting with clinically meaningful ILD and dcSSc was observed.Overall 8-year survival for males was 59% (95% CI 40% to 73%) and for females 89% (95% CI 82% to 93%). Eight-year survival showed a trend for improvement over time, and was always worse in males.
We observed a decrease in disease duration in Leiden CCISS cohort at cohort entry, possibly indicating more timely diagnosis of SSc. This could provide opportunities for early interventions. While symptom duration at presentation is longer in females, mortality is consistently higher in males, underlining the urge for sex-specific treatment and follow-up.
自 2009 年以来，荷兰确诊/疑似系统性硬化症（SSc）的患者可被转至莱顿系统性硬化症综合关怀（CCISS）队列。本研究旨在评估 SSc 的早期识别是否随时间推移而改善，以及疾病特征和生存是否随时间推移而改变。
纳入了符合美国风湿病学会/欧洲抗风湿病联盟 2013 年 SSc 标准的 643 例 SSc 患者，并根据队列入组年份分为三组：（1）2010-2013 年（n=229（36%）），（2）2014-2017 年（n=207（32%））和（3）2018-2021 年（n=207（32%））。比较了疾病持续时间、间质性肺病（ILD）、指溃疡（DU）、弥漫性皮肤 SSc（dcSSc）、抗拓扑异构酶（ATA）和抗着丝点（ACA）抗体以及从疾病发病到生存的变量在入组组之间的差异，包括按性别和自身抗体进行的分层分析。
随着时间的推移，男性和女性从疾病症状出现到队列入组的时间间隔缩短，但女性的时间间隔始终长于男性。出现 DU 的患者比例减少，尤其是 ACA+SSc 患者。几乎没有 ACA+患者出现ILD，而在 ATA+患者中，2010-2013 年这一比例为 25%，2018-2021 年降至 19%。出现有临床意义的ILD 和 dcSSc 的患者比例减少。男性的 8 年总生存率为 59%（95%CI 40%至 73%），女性为 89%（95%CI 82%至 93%）。8 年生存率随时间呈上升趋势，但男性始终较差。
我们观察到莱顿 CCISS 队列的入组疾病持续时间缩短，这可能表明 SSc 的诊断更加及时。这为早期干预提供了机会。虽然女性在就诊时的症状持续时间较长，但男性的死亡率始终较高，这强调了针对男性的特定治疗和随访的必要性。
