Bandeira Matilde, Silvério-António Manuel, Khmelinskii Nikita, Fonseca João E, Romão Vasco C
Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal.
Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisboa, Portugal.
Rheumatol Adv Pract. 2024 Mar 6;8(2):rkae035. doi: 10.1093/rap/rkae035. eCollection 2024.
Systemic extraglandular involvement in SS has been reported in one-third of patients but may be more frequent. We aimed to evaluate systemic disease prevalence at baseline and throughout follow-up and find its predictors.
We conducted a retrospective cohort study including SS patients followed in a tertiary centre. The cumulative EULAR SS disease activity index (ESSDAI) was calculated by adding each domain's maximum score throughout follow-up. We identified independent predictors of systemic involvement (ESSDAI ≥1 at baseline and/or follow-up) through logistic regression modelling. A survival analysis was conducted to identify predictors of new/worsening ESSDAI domains.
A total of 216 patients were included, most of whom had systemic involvement (86%), frequently at diagnosis (76%). Biological (53%) and articular ESSDAI domains (44%) were most commonly involved, but all were affected at least once. Around half of the patients with baseline systemic disease developed an additional/worsening domain throughout follow-up. Although most patients had low disease activity at baseline, 60% eventually reached moderately active disease. Younger age at diagnosis [odds ratio (OR) 0.95 (95% CI 0.91, 0.99)], a positive minor salivary gland biopsy [OR 4.08 (95% CI 1.40, 11.86)] and RF [OR 4.67 (95% CI 1.52, 14.33)] were independent predictors of systemic involvement. Patients with baseline constitutional involvement [hazard ratio (HR) 2.23 (95% CI 1.13, 4.40)] and RF [HR 1.89 (95% CI 1.20, 3.00)] were more likely to develop new/worsening systemic disease activity.
Systemic involvement is seen in most SS patients. Younger and RF and salivary gland biopsy-positive patients are at higher risk of systemic disease. Around half of patients with systemic involvement experienced aggravated disease over time, especially those with constitutional involvement or RF.
据报道，三分之一的干燥综合征（SS）患者存在系统性腺外受累情况，但实际可能更为常见。我们旨在评估基线及整个随访期间系统性疾病的患病率，并找出其预测因素。
我们进行了一项回顾性队列研究，纳入在三级中心接受随访的SS患者。通过将随访期间每个领域的最高分相加来计算累积欧洲抗风湿病联盟干燥综合征疾病活动指数（ESSDAI）。我们通过逻辑回归模型确定系统性受累（基线和/或随访时ESSDAI≥1）的独立预测因素。进行生存分析以确定新出现/病情加重的ESSDAI领域的预测因素。
共纳入216例患者，其中大多数有系统性受累（86%），常在诊断时出现（76%）。最常受累的是生物学（53%）和关节ESSDAI领域（44%），但所有领域至少有一次受累情况。约一半基线有系统性疾病的患者在整个随访期间出现了额外的/病情加重的领域。尽管大多数患者基线时疾病活动度较低，但60%的患者最终达到中度活动疾病状态。诊断时年龄较小[比值比（OR）0.95（95%置信区间0.91，0.99）]、小唾液腺活检阳性[OR 4.08（95%置信区间1.40，11.86）]和类风湿因子（RF）阳性[OR 4.67（95%置信区间1.52，14.33）]是系统性受累的独立预测因素。基线时有全身症状受累的患者[风险比（HR）2.23（95%置信区间1.13，4.40）]和RF阳性的患者[HR 1.89（95%置信区间1.20，3.00）]更有可能出现新的/病情加重的系统性疾病活动。
大多数SS患者存在系统性受累情况。年龄较小、RF阳性和唾液腺活检阳性的患者系统性疾病风险较高。约一半有系统性受累的患者随着时间推移病情加重，尤其是那些有全身症状受累或RF阳性的患者。
