Rheumatology Department, Leiden University Medical Center, Leiden, The Netherlands.
Medicine and Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy.
Rheumatology (Oxford). 2021 Aug 2;60(8):3646-3655. doi: 10.1093/rheumatology/keaa827.
In SSc patients, disease specific determinants that influence health-related quality of life (HRQoL) over time have not been described. We aim to, in patients with SSc, (i) evaluate if and how HRQoL changes over time, and (ii) assess how different SSc domains and functional impairments contribute to changes in HRQoL over time.
All SSc patients from the Leiden SSc cohort were included; patients with disease duration <24 months were classified as incident cases. HRQoL was assessed prospectively on an annual basis using the EQ-5D and the SF36. To assess baseline associations between clinical characteristics and HRQoL, linear regressions were performed. To identify possible associations between SSc characteristics and HRQoL change over time, linear mixed models were performed in both incident and prevalent cases.
In total, 492 SSc patients were included (n = 202 incident cases), with a median follow-up duration of 3.4 years. At baseline, presence of organ involvement was independently associated with a worse SF36 physical component score and lower EQ-5D score. Over time, gastrointestinal symptoms, Raynaud and digital ulcers were independently associated with deterioration of HRQoL in both incident and prevalent cases. In prevalent cases, pulmonary arterial hypertension (PAH) was associated with a decrease in HRQoL over time. Worse functioning as measured by six-min walking distance, mouth-opening, finger-to-palm distance and grip-strength contributed significantly to deterioration of HRQoL over time.
In SSc, key clinical burdens that contribute to worsening of HRQoL over time include digital ulcers, Raynaud and gastrointestinal involvement. In addition, PAH is a significant burden in prevalent disease.
在系统性硬化症（SSc）患者中，目前尚未描述随时间推移影响健康相关生活质量（HRQoL）的疾病特异性决定因素。我们的目的是，在 SSc 患者中：（i）评估 HRQoL 是否以及如何随时间变化，（ii）评估不同的 SSc 域和功能障碍如何导致 HRQoL 随时间的变化。
纳入莱顿 SSc 队列中的所有 SSc 患者；疾病持续时间<24 个月的患者被归类为新发病例。使用 EQ-5D 和 SF36 每年进行前瞻性 HRQoL 评估。为了评估基线临床特征与 HRQoL 之间的关联，进行了线性回归。为了确定 SSc 特征与随时间变化的 HRQoL 之间的可能关联，在新发和现患病例中均进行了线性混合模型分析。
共纳入 492 例 SSc 患者（n=202 例新发病例），中位随访时间为 3.4 年。基线时，器官受累的存在与 SF36 生理成分评分和 EQ-5D 评分较低独立相关。随时间推移，胃肠道症状、雷诺现象和指端溃疡与新发和现患病例 HRQoL 的恶化独立相关。在现患病例中，肺动脉高压（PAH）与随时间推移 HRQoL 的下降相关。六分钟步行距离、张口度、指掌距离和握力等功能越差，HRQoL 随时间恶化的程度越大。
在 SSc 中，随时间推移导致 HRQoL 恶化的主要临床负担包括指端溃疡、雷诺现象和胃肠道受累。此外，PAH 在现患疾病中是一个重要的负担。
