Aryanian Zeinab, Balighi Kamran, Azizpour Arghavan, RafieeTehrani Niyousha, Khayyat Azadeh, Hatami Parvaneh
Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Razi Hospital, Vahdat-e-Eslami St., Tehran, 1199663911, Iran.
Department of Dermatology, School of Medicine Razi Hospital, Tehran University of Medical Sciences, Tehran, 1199663911, Iran.
Arch Dermatol Res. 2025 Mar 14;317(1):562. doi: 10.1007/s00403-025-04025-0.
Some patients with pemphigus continue to have elevated levels of desmoglein autoantibodies (Dsg Ab) even after achieving remission. Thus, it is crucial to gain a deeper understanding of these patients' demographics and clinical patterns to improve prognosis, prevent complications, and devise individualized management strategies. This study utilized electronic patient records from a dermatology registry, focusing on PV and PF patients (diagnosed based on clinical and histopathologic diagnosis which included DIF) who achieved complete remission. We analyzed demographic and clinical data, including age, gender, BMI, disease type, lesion onset location, disease duration, test results, anti-desmoglein levels, and previous treatments. For comparative analysis, patients were categorized into two groups based on their Dsg Ab levels (high vs. normal) 3 months post-remission. Of the 80 patients evaluated, 57.5% were female, averaging 48.66 years. Significant gender differences were observed in Dsg Ab1 antibody levels, with males more likely to test sustained positive (P = 0.037), though no such difference was found for Dsg Ab 3 (P = 0.167). No significant differences in age, BMI, or history of other diseases were found between patients with high versus normal Dsg Abs post-recovery. The average time to complete recovery was 27.34 months, with no significant difference in recovery time or relapse rates based on antibody levels. However, there was a notable difference in some variables, including PDAI score, mucosal involvement, the average dose of prednisolone after achieving complete remission and occurrence of infectious complications (only regarding Dsg Ab 3) between the two groups (P < 0.05). The study underscores the heterogeneity in the post-remission course of pemphigus patients regarding the persistence of high Dsg Ab levels in some individuals. These findings emphasize the need for a tailored approach to management and monitoring of patients. Further research into the mechanisms driving these differences could pave the way for more effective treatments and prognostic tools in pemphigus care.
一些天疱疮患者即使在病情缓解后，其桥粒芯糖蛋白自身抗体（Dsg Ab）水平仍持续升高。因此，深入了解这些患者的人口统计学特征和临床模式对于改善预后、预防并发症以及制定个体化管理策略至关重要。本研究利用了皮肤科登记处的电子病历，重点关注达到完全缓解的寻常型天疱疮（PV）和落叶型天疱疮（PF）患者（基于包括直接免疫荧光法（DIF）在内的临床和组织病理学诊断）。我们分析了人口统计学和临床数据，包括年龄、性别、体重指数（BMI）、疾病类型、皮损起始部位、病程、检测结果、抗桥粒芯糖蛋白水平以及既往治疗情况。为了进行比较分析，患者在缓解后3个月根据其Dsg Ab水平分为两组（高水平组与正常水平组）。在评估的80例患者中，57.5%为女性，平均年龄48.66岁。在Dsg Ab1抗体水平上观察到显著的性别差异，男性更有可能检测为持续阳性（P = 0.037），而Dsg Ab 3则未发现此类差异（P = 0.167）。恢复后Dsg Ab水平高的患者与正常患者在年龄、BMI或其他疾病史方面未发现显著差异。完全恢复的平均时间为27.34个月，基于抗体水平的恢复时间或复发率无显著差异。然而，两组在一些变量上存在显著差异，包括天疱疮疾病面积和严重程度指数（PDAI）评分、黏膜受累情况、完全缓解后泼尼松龙的平均剂量以及感染性并发症的发生情况（仅针对Dsg Ab 3）（P < 0.05）。该研究强调了天疱疮患者缓解后病程的异质性，即部分个体中高Dsg Ab水平持续存在。这些发现强调了对患者进行个体化管理和监测的必要性。对导致这些差异的机制进行进一步研究可为天疱疮治疗中更有效的治疗方法和预后工具铺平道路。
