The University of Melbourne, Victoria, and St. Vincent's Hospital Melbourne, Victoria, Australia.
St. Vincent's Hospital Melbourne, Victoria, Australia.
Clin Exp Rheumatol. 2024 Aug;42(8):1581-1589. doi: 10.55563/clinexprheumatol/9erk5j. Epub 2024 Aug 2.
To identify the trajectories and clinical associations of functional disability in systemic sclerosis (SSc).
Australian Scleroderma Cohort Study (ASCS) participants meeting ACR/EULAR criteria for SSc recruited within 5 years of disease onset, with ≥2 Health Assessment Questionnaire-Disability Index (HAQ-DI) scores were included. Group based trajectory modelling (GBTM) was used to identify the number and shape of HAQ-DI trajectories. Between group comparisons were made using the chi-squared test, two-sample t-test or Wilcoxon rank-sum test as appropriate. Multiple logistic regression was used to identify features associated with trajectory group membership. Survival analyses were performed using Kaplan Meier and Cox proportional hazard modelling.
We identified two HAQ-DI trajectory groups within 426 ASCS participants with incident SSc: low-stable disability (n=221, 52%), and high-increasing disability (n=205, 48%). Participants with high-increasing disability were older at disease onset, more likely to have diffuse SSc (dcSSc), cardiopulmonary disease, multimorbidity, digital ulcers, and gastrointestinal involvement (all p≤0.01), as was use of immunosuppression (p<0.01). Multimorbidity was associated with high-increasing trajectory group membership (OR3.1, 95%CI1.1-8.8, p=0.04); independently, multiple SSc features were also strongly associated including dcSSc (OR2.3, 95%CI1.3-4.2, p<0.01), proximal weakness (OR7.3, 95%CI2.0-27.1, p<0.01) and joint contractures (OR2.7, 95%CI1.3-5.3, p<0.01). High-increasing physical disability was associated with an almost two-fold increased risk of mortality (HR1.9, 95%CI1.0-3.8, p=0.05), and higher symptom burden.
Two trajectories of functional disability in SSc were identified. Those with high-increasing functional disability had a distinct clinical phenotype and worse survival compared to those with low-stable functional disability. These data highlight the pervasive nature of physical disability in SSc, and its prognostic importance.
确定系统性硬化症（SSc）患者功能障碍的轨迹和临床相关性。
纳入符合 ACR/EULAR 系统性硬化症标准、发病后 5 年内入组的澳大利亚硬皮病队列研究（ASCS）参与者，且至少有 2 次健康评估问卷残疾指数（HAQ-DI）评分。采用基于群组的轨迹建模（GBTM）来确定 HAQ-DI 轨迹的数量和形状。使用卡方检验、两样本 t 检验或 Wilcoxon 秩和检验进行组间比较，根据情况选择合适的检验方法。采用多因素逻辑回归分析确定与轨迹组归属相关的特征。使用 Kaplan-Meier 和 Cox 比例风险模型进行生存分析。
我们在 426 例有新发 SSc 的 ASCS 参与者中发现了 2 种 HAQ-DI 轨迹组：低稳定残疾组（n=221，52%）和高递增残疾组（n=205，48%）。高递增残疾组的发病年龄较大，更有可能患有弥漫性 SSc（dcSSc）、心肺疾病、多种合并症、指溃疡和胃肠道受累（均 p≤0.01），且更有可能使用免疫抑制剂（p<0.01）。多种合并症与高递增轨迹组归属相关（OR3.1，95%CI1.1-8.8，p=0.04）；独立地，多个 SSc 特征也与高递增轨迹组归属强烈相关，包括 dcSSc（OR2.3，95%CI1.3-4.2，p<0.01）、近端肌无力（OR7.3，95%CI2.0-27.1，p<0.01）和关节挛缩（OR2.7，95%CI1.3-5.3，p<0.01）。高递增的身体残疾与死亡风险增加近两倍相关（HR1.9，95%CI1.0-3.8，p=0.05），且与更高的症状负担相关。
确定了 SSc 中 2 种功能障碍轨迹。与低稳定功能障碍相比，高递增功能障碍患者具有明显不同的临床表型和更差的生存。这些数据突出了 SSc 中身体残疾的普遍性质及其预后重要性。
