天疱疮主要自身抗体及其检测临床意义研究进展

doi:10.16150/j.1671-2870.2022.05.017

摘要/Abstract

摘要：

天疱疮是一种严重的自身免疫性疾病，主要损害皮肤及黏膜的屏障功能。该病是由患者体内产生的针对细胞间黏附分子等结构的自身抗体导致，其中主要产生致病作用的抗体是针对桥粒芯蛋白（desmogelin，Dsg）1和Dsg3的抗体。这2种抗体主要通过空间位阻效应及抗原抗体结合之后的信号转导两方面机制致病，而抗Dsc抗体、抗斑蛋白家族抗体、抗乙酰胆碱受体抗体等也在致病过程中起着重要的协同作用。随着免疫荧光、酶联免疫吸附试验（enzyme-linked immunosorbent assays，ELISA）等技术的普及，抗体检测已成为天疱疮临床诊断、病程监测的重要方法。新近临床研究提示，抗Dsg1、Dsg3抗体滴度与天疱疮病情活动程度间存在相关性，同时可监测病情的复发及指导临床用药。

关键词: 天疱疮, 自身抗体, 桥粒芯蛋白, 疗效监测

Abstract:

Pemphigus is a severe autoimmune disease causing damage of epidermis and mucosa. Patients can produce autoantibodies against structures like intercellular adhesion, which leads to disease. Main pathogenic antibodies include anti-Gsg1 and anti-Gsg3 which cause disease primarily through steric hindrance and signal transduction after antigen-antibody binding. Other antibodies such as anti-Dsc, anti-plakin family, anti-acetylcholine receptor etc. also play an important synergistic effect in the pathogenic process. With the popularization of immunofluorescence, ELISA and other technologies, antibody detection has become an important method for clinical diagnosis and monitoring of pemphigus. Couples of clinical studies have shown that the titers of anti-Dsg1 and Dsg3 antibodies are related to the activity of pemphigus disease, which can also indicate the recurrence of the disease and guide clinical medication. More mechanism research and clinical exploration of pemphigus autoantibodies will help us to cognize the disease further.

Key words: Pemphigus, Autoantibody, Desmogelin, Efficacy monitoring

中图分类号:

R758.66

王麒钧, 朱海琴, 潘萌. 天疱疮主要自身抗体及其检测临床意义研究进展[J]. 诊断学理论与实践, 2022, 21(05): 638-643.

WANG Qijun, ZHU Haiqin, PAN Meng. Major autoantibodies in pemphigus: detection and clinical significance[J]. Journal of Diagnostics Concepts & Practice, 2022, 21(05): 638-643.

图/表 1

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检测方法	检测底物	疾病	灵敏度	特异度
直接免疫荧光	皮肤样本	PV/PF	82%~91%	98%
间接免疫荧光	猴食道	PV/PF	81%~100%	89%~100%
	鼠膀胱	PNP	74%	99%~100%
酶联免疫吸附试验	Dsg1	PF	96%~100%	96%~100%
	Dsg3	PV	85%~100%	96%~100%
	Envoplakin	PNP	63%~83%	91%~98%
	Periplakin	PNP	60%	无数据
BIOCHIP	猴食道	PV/PF	83%~100%	89%~100%
	重组Dsg1	PV/PF	38%~90%	99%
	重组Dsg3	PV	87%~100%	97%~100%