8例缓解期高滴度血清抗体的天疱疮患者临床分析

Clinical Analysis of Eight Cases of Pemphigus with High Titers of Anti-desmoglein Antibodies in Remission

目的评估缓解期血清抗桥粒芯蛋白(Dsg)抗体呈现高滴度的天疱疮患者的临床表现、诊治过程及实验室检查结果,为此类患者的治疗提供依据。方法回顾性分析2013年1月至2020年9月皮肤科诊治的天疱疮患者临床资料,收集确诊为天疱疮并且缓解期血清抗Dsg抗体水平(酶联免疫吸附测定法测定)≥150 U/ml或较初始治疗时下降≤20%(以最大检测上限150 U/ml计算)的患者资料。结果在此期间收治并获得随访的134例天疱疮患者中,共有8例患者符合纳入标准,随访时长21~85个月,持续缓解时间18~70个月,均以每日口服≤10 mg泼尼松相当量糖皮质激素维持治疗,血清抗Dsg抗体均呈高滴度。8例患者首诊时Dsg-1抗体阳性和Dsg-3抗体阳性者各为7例,其中2例患者分别在病情缓解后19和21个月Dsg-1抗体仍为高滴度,5例患者在病情缓解的18~70个月Dsg-3抗体持续保持高滴度,1例患者两种抗体均呈高滴度状态,以Dsg-1抗体更为明显。Dsg-1和Dsg-3抗体均持续高滴度的患者在19个月的缓解期后出现复发,余患者仍处于临床缓解状态。结论天疱疮患者治疗后,部分患者抗体滴度呈现高水平状态,以Dsg-3抗体持续不转阴更常见,且与复发无明显相关性,而Dsg-1抗体不下降较少见,患者复发可能性较Dsg-3不下降者大。对于这类临床长期缓解但抗体呈高滴度的天疱疮患者,应注意根据抗体类型并结合患者实际情况调整糖皮质激素用量。对于Dsg-1抗体持续高水平的患者,激素减量应适度放缓。

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.