Jin Xin Xing, Li Si Zhe, Zuo Ya Gang, Jin Hong Zhong
Department of Dermatology,Translational Medicine Center,National Clinical Research Center for Dermatologic and Immunologic Diseases,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2021 Apr 28;43(2):166-172. doi: 10.3881/j.issn.1000-503X.13452.
Objective To investigate the clinical manifestations,diagnosis,treatment,and laboratory examination characteristics of 8 pemphigus patients with high titers of anti-desmoglein antibodies in remission. Methods A retrospective study was conducted for the pemphigus patients diagnosed and treated in the department of dermatology from January 2013 to September 2020.The patients should have the serum anti-desmoglein antibodies ≥150 U/ml in remission or the antibody levels dropped less than 20%(calculated based on the maximum detection limit of 150 U/ml)of their initial ones detected before treatment,and the clinical and laboratory data of patients eligible for the inclusion criteria were collected. Results Among the 134 pemphigus patients with available follow-up data during this period,a total of 8 patients met the criteria,with the follow-up period of 21-85 months and the remission duration of 18-70 months.They all received less than or equal to 10 mg/d prednisone and had high titers of anti-desmoglein antibodies.At their first visit,the number of patients with positive anti-desmoglein 1/desmoglein 3 antibodies was 7.Two patients still had high titers of anti-desmoglein 1 antibodies 19 months and 21 months after they achieved remission,and 5 patients had high titers of anti-desmoglein 3 antibodies in 18-70 months.There was one patient showing high titers of both antibodies,especially for anti-desmoglein 1 antibodies.This patient relapsed after 19 months' remission while other patients were still in clinical remission. Conclusions Some pemphigus patients showed persistent high titers of anti-desmoglein antibodies in remission.Anti-desmoglein 3 antibodies were more common to keep positive,while high titer of anti-desmoglein 1 antibodies was less observed.The high titer of anti-desmoglein 1 antibodies had a correlation with recurrence.For the pemphigus patients with long-term clinical remission but high antibody titer,the dosages of corticosteroids should be adjusted carefully according to their actual clinical manifestations and the positive antibody type.For the patients with high titer of anti-desmoglein 1 antibodies,the dosage reduction of corticosteroids should be appropriately slower.
目的 探讨8例缓解期抗桥粒芯糖蛋白抗体高滴度的天疱疮患者的临床表现、诊断、治疗及实验室检查特点。方法 对2013年1月至2020年9月在皮肤科确诊并治疗的天疱疮患者进行回顾性研究。患者缓解期血清抗桥粒芯糖蛋白抗体≥150 U/ml，或抗体水平较治疗前首次检测的初始值下降不到20%（按最大检测限150 U/ml计算），收集符合纳入标准患者的临床和实验室数据。结果 在此期间有随访数据的134例天疱疮患者中，共有8例符合标准，随访时间为21 - 85个月，缓解期为18 - 70个月。他们均接受≤10 mg/d的泼尼松治疗且抗桥粒芯糖蛋白抗体滴度高。初诊时，抗桥粒芯糖蛋白1/桥粒芯糖蛋白3抗体阳性的患者有7例。2例患者缓解19个月和21个月后抗桥粒芯糖蛋白1抗体仍为高滴度，5例患者在18 - 70个月抗桥粒芯糖蛋白3抗体为高滴度。有1例患者两种抗体均为高滴度，尤其是抗桥粒芯糖蛋白1抗体。该患者缓解19个月后复发，而其他患者仍处于临床缓解状态。结论 部分天疱疮患者缓解期抗桥粒芯糖蛋白抗体持续高滴度。抗桥粒芯糖蛋白3抗体持续阳性更常见，而抗桥粒芯糖蛋白1抗体高滴度较少见。抗桥粒芯糖蛋白1抗体高滴度与复发有关。对于临床长期缓解但抗体滴度高的天疱疮患者，应根据其实际临床表现及抗体阳性类型谨慎调整糖皮质激素剂量。对于抗桥粒芯糖蛋白1抗体高滴度的患者，糖皮质激素减量应适当放缓。
