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免疫预测寻常型天疱疮(PV)患者临床缓解后的复发。

Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission.

机构信息

Autoimmune Bullous Diseases Research Center, Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran.

Autoimmune Bullous Diseases Research Center, Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

J Am Acad Dermatol. 2016 Jun;74(6):1160-5. doi: 10.1016/j.jaad.2015.10.051. Epub 2016 Feb 17.

DOI:10.1016/j.jaad.2015.10.051
PMID:26896293
Abstract

BACKGROUND

Pemphigus vulgaris (PV) is characterized by multiple relapses, occurring especially in patients on minimal therapy or off therapy.

OBJECTIVE

To identify immunologic predictors (anti-desmoglein [Dsg] 1 and 3 antibodies; direct immunofluorescence [DIF]) for relapse in PV patients.

METHODS

Eighty-nine patients in complete clinical remission for at least 6 months and receiving less than or equal to 10 mg prednisolone daily and no immunosuppressive drugs were evaluated using DIF (n=89) and Dsg ELISA (n=46). They were followed until relapse or for at least 18 months.

RESULTS

DIF was positive in 44 of 89 patients (49.5%); anti-Dsg 3 antibodies were detected in 18 of 46 patients (39.1%) and anti-Dsg 1 antibodies were detected in 4 of 46 patients (8.7%). Relapse occurred in 38 patients (42.7%). Mean relapse-free time was significantly shorter in anti-Dsg 3-positive patients compared to anti-Dsg 3- negative patients (P = .015) and in DIF-positive patients compared to DIF-negative patients (P = .047), but not in anti-Dsg 1- positive patients compared to anti-Dsg 1-negative patients (P = .501). Sensitivity and predictive values of neither of these tests were high.

LIMITATIONS

Small number of anti-Dsg 1-positive patients and use of conventional ELISA.

CONCLUSION

Positive anti-Dsg 3 ELISA and, to a lesser degree, positive DIF are predictors of relapse in PV patients in clinical remission. Decision on discontinuing treatment should be based on the results of these tests as well as on clinical findings.

摘要

背景

寻常型天疱疮(PV)的特征是多次复发,尤其是在接受最低剂量治疗或停药的患者中。

目的

确定寻常型天疱疮患者复发的免疫预测因子(抗桥粒芯糖蛋白[Dsg]1 和 3 抗体;直接免疫荧光[DIF])。

方法

对 89 例至少 6 个月完全缓解且接受低于或等于 10mg 泼尼松龙/日和无免疫抑制剂治疗的患者进行 DIF(n=89)和 Dsg ELISA(n=46)评估。随访至复发或至少 18 个月。

结果

44 例/89 例(49.5%)DIF 阳性;18 例/46 例(39.1%)检测到抗 Dsg 3 抗体,4 例/46 例(8.7%)检测到抗 Dsg 1 抗体。38 例患者(42.7%)复发。与抗 Dsg 3 阴性患者相比,抗 Dsg 3 阳性患者的无复发生存时间更短(P=.015),与 DIF 阴性患者相比,DIF 阳性患者的无复发生存时间更短(P=.047),而与抗 Dsg 1 阴性患者相比,抗 Dsg 1 阳性患者的无复发生存时间无差异(P=.501)。这些检测的敏感性和预测值均不高。

局限性

抗 Dsg 1 阳性患者数量较少,且使用传统 ELISA。

结论

在临床缓解的寻常型天疱疮患者中,抗 Dsg 3 ELISA 阳性,且程度较轻时,DIF 阳性是复发的预测因子。停止治疗的决定应基于这些测试的结果以及临床发现。

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