Zhou Yuxi, Wang Yiyi, Wang Mi, Zhou Xingli, Luo Limei, Yan Wei, Li Wei
Department of Dermatology & Venerology, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, China.
Department of Dermatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Front Immunol. 2025 May 13;16:1557556. doi: 10.3389/fimmu.2025.1557556. eCollection 2025.
Anti-desmoglein (Dsg) antibodies are well-established markers correlated with clinical phenotype and disease severity in pemphigus vulgaris (PV). However, elevated anti-Dsg antibody levels have been observed in some patients during clinical remission (CR). This study aimed to identify clinical characteristics and risk factors in PV patients with elevated anti-Dsg antibodies after achieving CR.
We conducted a cohort study based on the prospective registry database of autoimmune bullous diseases patients at West China Hospital between April 2016 and March 2022. PV patients with at least 12 months of follow-up were enrolled. The pemphigus disease area index (PDAI) and anti-Dsg antibody titers were measured at baseline and 1, 3, 6, 9, 12 months during follow-up. Univariate, multivariate analyses and receiver operating characteristics (ROC) curves were performed to identify associated factors with persistent antibody positivity and optimal cut-off values respectively. The primary outcome was the persistent positivity of antibodies against Dsg after achieving CR.
Among 239 PV patients enrolled in this study, 118 (49%) achieved CR. Cataracts were identified as an independent risk factor for persistent anti-Dsg1 positivity after CR. Higher baseline anti-Dsg3 antibody titers and PDAI scores were significant predictors of increased anti-Dsg3 levels post-CR, with gender also being a contributing factor. ROC analysis determined a cut-off value of 157.4 U/mL for anti-Dsg3 with 56.3% sensitivity and 82.6% specificity.
The presence of Cataracts may indicate persistent anti-Dsg1 positivity after CR, while elevated anti-Dsg3 titers and PDAI scores at baseline may predict sustained elevated anti-Dsg3 post-CR.
抗桥粒芯糖蛋白（Dsg）抗体是寻常型天疱疮（PV）中与临床表型和疾病严重程度相关的公认标志物。然而，在一些患者临床缓解（CR）期间观察到抗Dsg抗体水平升高。本研究旨在确定CR后抗Dsg抗体升高的PV患者的临床特征和危险因素。
我们基于2016年4月至2022年3月期间华西医院自身免疫性大疱性疾病患者的前瞻性登记数据库进行了一项队列研究。纳入至少随访12个月的PV患者。在基线以及随访期间的第1、3、6、9、12个月测量天疱疮疾病面积指数（PDAI）和抗Dsg抗体滴度。分别进行单因素、多因素分析和受试者工作特征（ROC）曲线分析，以确定与抗体持续阳性相关的因素和最佳截断值。主要结局是达到CR后抗Dsg抗体的持续阳性。
在本研究纳入的239例PV患者中，118例（49%）达到CR。白内障被确定为CR后抗Dsg1持续阳性的独立危险因素。较高的基线抗Dsg3抗体滴度和PDAI评分是CR后抗Dsg3水平升高的重要预测因素，性别也是一个影响因素。ROC分析确定抗Dsg3的截断值为157.4 U/mL，敏感性为56.3%，特异性为82.6%。
白内障的存在可能表明CR后抗Dsg1持续阳性，而基线时抗Dsg3滴度和PDAI评分升高可能预测CR后抗Dsg3持续升高。
