天疱疮特异性抗体亚型与疾病活动的相关性研究

目的 探讨天疱疮患者抗桥粒芯糖蛋白（Dsg）1和Dsg3抗体亚型与疾病活动的相关性。方法 收集47例天疱疮患者血清，ELISA检测其特异性抗Dsg1和Dsg3抗体及亚型，分析抗体滴度及亚型与疾病活动的相关性。结果 抗Dsg1和Dsg3抗体类别与天疱疮临床类型有关，17例皮肤黏膜同时受累的患者中有14例（82.4%）同时存在两种抗体；16例仅皮肤受累的患者中有15例（93.7%）存在抗Dsg1抗体，仅1例（6.3%）存在抗Dsg3抗体；6例仅黏膜受累的患者只存在抗Dsg3抗体，阳性率为100％。随着病情的加重，抗Dsg1和Dsg3抗体滴度有上升趋势，但该趋势与疾病的严重度并不完全平行。而抗体亚型则和疾病活动相关，活动期以特异性IgG4亚型为主，稳定期以IgG1亚型为主。抗Dsg1阳性者，活动期特异性IgG4和IgG1抗体吸光度（A）值分别为1.92 ± 1.21和0.60 ± 0.61，IgG4/IgG1 > 1；稳定期特异性IgG4和IgG1抗体A值分别为0.03 ± 0.02和0.22 ± 0.11，IgG4/IgG1 < 1；抗Dsg3阳性者，活动期特异性IgG4和IgG1抗体A值分别为2.35 ± 2.17和1.84 ± 1.16，IgG4/IgG1 > 1；稳定期特异性IgG4和IgG1抗体A值分别为0.15 ± 0.16和1.05 ± 0.77，IgG4/IgG1 < 1。结论 天疱疮特异性抗体亚型与疾病活动密切相关，用ELISA检测天疱疮患者血清中抗Dsg1和Dsg3抗体类型、特异性抗体亚型及滴度，可更好地辅助诊断疾病、监测疾病活动。

Correlation between disease activity and pemphigus-specific antibody subclasses in patients with pemphigus

Objective To assess the correlation between the subclasses of antibodies against desmoglein (Dsg) 1 and Dsg3 and disease activity in patients with pemphigus. Methods Sera were collected from 47 patients with pemphigus, and ELISA was performed to determine the titers and subclasses of antibodies against Dsg1 and Dsg3. The correlation of antibody titers and subclasses with disease activity was assessed.Results Clinical phenotype was associated with antibody profiles in these patients with pemphigus. Of 17 patients with mucocutaneous involvement, 14 (82.4%) had both anti-Dsg3 and anti-Dsg1 antibodies; of 16 patients with cutaneous involvement, 15 (93.7%) had anti-Dsg1 antibody, only 1 (6.3%) developed anti-Dsg3 antibody; of 6 patients with mucosal involvement, all ( 100% ) had only anti-Dsg3 antibody. The serum levels of antibodies against Dsg1 and Dsg3 were increased, but not in parallel with the disease severity in these patients.Moreover, the subclasses of antibodies were correlated with disease severity. IgG4 subclass antibodies against Dsg1 and Dsg3 predominated in patients with pemphigus at active stage, whereas IgG1 subclass in those at remission stage. The serum levels (expressed as absorbance value) of IgG4 and IgG1 subclass antibodies were 1.92 ± 1.21 and 0.60 ± 0.61 respectively, with the ratio of IgG4 to IgG1 more than 1, in patients with antiDsg1 antibodies at active stage, 0.03 ± 0.02 and 0.22 ± 0.11 respectively with the ratio of IgG4 to IgG1 less than 1, in those at remission stage, 2.35 ± 2.17 and 1.84 ± 1.16 respectively with the ratio of IgG4 to IgG1 more than 1 in patients with anti-Dsg3 antibodies at active stage, 0.15 ± 0.16 and 1.05 ± 0.77 respectively with the ratio of IgG4 to IgG1 less than 1 in those at remission stage. Conclusions The subclasses of pemphigus-specific antibodies are closely correlated with the disease severity in patients with pemphigus. The detection of subclasses and titers of anti-Dsg1 and anti-Dsg3 antibodies may aid in the diagnosis of and monitoring of disease severity in pemphigus.