Kao Wei-Chen, Hu Ya-Chiao, Lee Jyh-Hong, Wang Li-Chieh, Lin Yu-Tsan, Yang Yao-Hsu, Chiang Bor-Luen, Yu Hsin-Hui
Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan.
Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei, Taiwan.
Pediatr Rheumatol Online J. 2025 Apr 1;23(1):36. doi: 10.1186/s12969-025-01084-5.
Systemic lupus erythematosus (SLE) is a multi-systemic autoimmune disease that causes inflammation of the serosa (serositis). This retrospective study aimed to evaluate the clinical characteristics of serositis in childhood-onset SLE (cSLE) and analyze its association with long-term outcomes.
We retrospectively reviewed the medical records of patients with cSLE diagnosed at a medical center in Taiwan, analyzing data collected from January 2002 to December 2022. We analyzed the clinical features of patients with serositis as pleuritis and/or pericarditis with at least a small effusion (> 0.5 cm in depth) on sonography or chest radiography. Cox proportional hazards regression was used to calculate the hazard ratios (HR) and 95% confidence intervals (CI) for the association between serositis and all-cause mortality.
185 patients with cSLE were enrolled, of whom 38 (20.54%) had serositis. Patients with serositis had a younger age at SLE diagnosis, a higher SLE Disease Activity Index 2000 score at serositis diagnosis, and an increased prevalence of lupus nephritis, central nervous system manifestations, end-stage renal disease (ESRD), a higher Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index score, and a higher mortality than that of patients without serositis. Multivariate Cox regression analysis showed that both serositis (hazard ratio [HR]: 5.585, confidence interval [CI]: 1.853-17.80) and ESRD (HR: 13.956; CI: 3.822-50.964) were associated with mortality risk. Kaplan-Meier survival curve analysis revealed that patients with both serositis and ESRD had the poorest 20-year survival rate. Patients with late-onset serositis (occurring 1 year after SLE diagnosis) had higher mortality rates than those with early-onset serositis.
Children with lupus serositis had higher disease activity, a higher prevalence of comorbidities, and mortality. Patients with both serositis, especially late-onset serositis, and ESRD had an increased risk of poor long-term survival.
系统性红斑狼疮（SLE）是一种多系统自身免疫性疾病，可导致浆膜炎症（浆膜炎）。这项回顾性研究旨在评估儿童期起病的系统性红斑狼疮（cSLE）中浆膜炎的临床特征，并分析其与长期预后的关联。
我们回顾性分析了台湾一家医疗中心确诊的cSLE患者的病历，分析了2002年1月至2022年12月收集的数据。我们分析了浆膜炎患者的临床特征，这些患者表现为胸膜炎和/或心包炎，超声或胸部X线检查至少有少量积液（深度>0.5厘米）。采用Cox比例风险回归分析来计算浆膜炎与全因死亡率之间关联的风险比（HR）和95%置信区间（CI）。
共纳入185例cSLE患者，其中38例（20.54%）有浆膜炎。有浆膜炎的患者SLE诊断时年龄更小，浆膜炎诊断时SLE疾病活动指数2000评分更高，狼疮性肾炎、中枢神经系统表现、终末期肾病（ESRD）的患病率增加，系统性红斑狼疮国际协作临床组（SLICC）/美国风湿病学会（ACR）损伤指数评分更高，且死亡率高于无浆膜炎的患者。多因素Cox回归分析显示，浆膜炎（风险比[HR]：5.585，置信区间[CI]：1.853 - 17.80）和ESRD（HR：1,3.956；CI：3.822 - 50.964）均与死亡风险相关。Kaplan - Meier生存曲线分析显示，同时患有浆膜炎和ESRD的患者20年生存率最差。迟发性浆膜炎（SLE诊断后1年出现）患者的死亡率高于早发性浆膜炎患者。
患有狼疮性浆膜炎的儿童疾病活动度更高，合并症患病率更高，且死亡率更高。同时患有浆膜炎，尤其是迟发性浆膜炎和ESRD的患者长期生存不良风险增加。
