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基于人口统计学、临床参数和复发倾向的印度和欧洲天疱疮患者差异:一项前瞻性的洲际队列研究结果。

Differences among Indian and European pemphigus patients based on demographics, clinical parameters and propensity for relapse: results of a prospective bicontinental cohort study.

机构信息

Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Department of Dermatology and Venereology, Faculty of Medicine, Medical University - Sofia, Bulgaria.

出版信息

Eur J Dermatol. 2023 Dec 1;33(6):642-647. doi: 10.1684/ejd.2023.4631.

DOI:10.1684/ejd.2023.4631
PMID:38465545
Abstract

Limited studies have explored pemphigus variations among different ethnic groups residing in their respective geographical locations. This bicontinental study aimed to compare clinical and immunological parameters in Indian and European pemphigus patients in complete remission, off therapy, or on minimal therapy. 105 patients (India, n= 75; Bulgaria, n=15; Greece, n=15) with pemphigus vulgaris (PV) or pemphigus foliaceous (PF) in complete remission on minimal therapy (n=64) or complete remission off therapy (n=41) were recruited. Demographic, clinical, and immunological parameters were compared. Indian patients were significantly younger, the maximal disease severity during the preceding active disease phase was significantly lower, and treatment duration until complete remission was significantly shorter, compared to European patients. European patients had significantly higher anti-Dsg3 serum levels and higher IgG positivity rate based on direct immunofluorescence microscopy at baseline. Furthermore, European patients revealed higher CD19, CD19+ CD27+ cell counts, compared with patients from India. Of note, none of the European patients (n=30) relapsed within the study period, in contrast to 29/75 (38.6%) Indian patients. Treatment strategies differed significantly between the two cohorts, with more frequent utilization of rituximab to achieve remission in the Indian cohort, while prednisolone was more widely used for maintaining remission in the European cohort. The observed heterogeneity of pemphigus among patients of different ethnicities in terms of demographics, clinical parameters, and propensity for relapse may be due to genetic background or different treatment strategies.

摘要

有限的研究探讨了居住在各自地理位置的不同种族群体中的天疱疮变异。这项两大陆研究旨在比较印度和欧洲天疱疮患者在完全缓解、停药或最低剂量治疗时的临床和免疫参数。招募了 105 名患有寻常型天疱疮(PV)或落叶型天疱疮(PF)的患者(印度,n=75;保加利亚,n=15;希腊,n=15),这些患者处于最低剂量治疗的完全缓解期(n=64)或停药后的完全缓解期(n=41)。比较了人口统计学、临床和免疫学参数。与欧洲患者相比,印度患者明显更年轻,在前一个活跃疾病阶段的最大疾病严重程度明显更低,达到完全缓解的治疗持续时间明显更短。欧洲患者的抗 Dsg3 血清水平明显更高,基线时直接免疫荧光显微镜检查的 IgG 阳性率也更高。此外,与印度患者相比,欧洲患者的 CD19 和 CD19+ CD27+细胞计数明显更高。值得注意的是,在研究期间,没有一名欧洲患者(n=30)复发,而印度患者中有 29/75(38.6%)复发。两个队列的治疗策略存在显著差异,印度队列更频繁地使用利妥昔单抗来实现缓解,而欧洲队列更广泛地使用泼尼松龙来维持缓解。不同种族患者在人口统计学、临床参数和复发倾向方面的天疱疮异质性可能归因于遗传背景或不同的治疗策略。

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引用本文的文献

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Antidesmoglein 1 and 3 serum IgG and positivity by direct immunofluorescence microscopy is associated with relapse in pemphigus in a prospective bicontinental study.在一项前瞻性的跨两大洲研究中,桥粒芯糖蛋白1和3血清IgG以及直接免疫荧光显微镜检查的阳性结果与天疱疮的复发相关。
JAAD Int. 2025 Jan 20;19:67-74. doi: 10.1016/j.jdin.2024.12.004. eCollection 2025 Apr.
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Consensus Based Indian Guidelines for the Management of Pemphigus Vulgaris and Pemphigus Foliaceous.基于共识的印度寻常型天疱疮和落叶型天疱疮管理指南。
Indian Dermatol Online J. 2024 Dec 26;16(1):3-24. doi: 10.4103/idoj.idoj_1059_24. eCollection 2025 Jan-Feb.