Analysis of clinical characteristics, prognosis and antibody pathogenicity of pemphigus patients positive for anti‐desmoglein IgG autoantibodies in remission: a retrospective cohort study
Analysis of clinical characteristics, prognosis and antibody pathogenicity of pemphigus patients positive for anti‐desmoglein IgG autoantibodies in remission: a retrospective cohort study
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抗桥粒芯糖蛋白IgG自身抗体阳性缓解期天疱疮患者临床特征、预后及抗体致病性分析：一项回顾性队列研究
DOI：
10.1111/jdv.17770
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发表时间：
2021
影响因子：
9.2
通讯作者：
Yamagami J.
中科院分区：
文献类型：
--
作者：
Zhao W.L.;Ishii K.;Egami S.;Xu Z.;Funakoshi T.;Takahashi H.;Tanikawa A.;Ishiko A.;Amagai M.;Yamagami J.
背景检测血清抗桥粒芯糖蛋白（Dsg）IgG自身抗体已被报道可用于评估天疱疮的疾病活动性。然而，以前的研究报告，抗Dsg自身抗体仍然检测到一些患者没有活动性天疱疮lesions.ObjectivesTo调查天疱疮患者的临床特征和抗体致病性的抗Dsg IgG自身抗体阳性的缓解期。方法我们回顾性调查天疱疮患者的临床缓解的历史谁访问了庆应义塾大学皮肤科在2019年和2020年。抗体致病性进行了评估珠aggregationassay.ResultsWhen患者被确认为已进入缓解期（PDAI = 0和PSL <$10 mg/天，2个月），血清抗Dsg自身抗体检测72 132例（54.5%，阳性组; PG），但未检测到60例（45.5%，阴性组; NG）。PG中33例患者的缓解期抗Dsg抗体滴度较活动期下降，这些患者的数据可用。PG组和NG组PSL降至5 mg/d的机会（P= 0.885）和复发率（P= 0.279）无差异，但PG组停用糖皮质激素的患者较少（P= 0.004）。与活动期相比，缓解期患者血清阻断Dsg/桥粒胶珠聚集的能力显着降低。然而，我们的研究结果显示，在缓解期的全血清中仍有致病活性的9例患者中的7例，大约等量的抗Dsg抗体在活动期和缓解期表现出类似的致病性.ConclusionsThis研究将提供指导的情况下，发现自身抗体是阳性的天疱疮患者在缓解或类固醇减少。
BackgroundThe detection of serum anti‐desmoglein (Dsg) IgG autoantibodies has been reported to be useful for assessment of disease activity in pemphigus. However, previous studies have reported that anti‐Dsg autoantibodies remain detectable in some patients without active pemphigus lesions.ObjectivesTo investigate the clinical characteristics and antibody pathogenicity of pemphigus patients positive for anti‐Dsg IgG autoantibodies in remission.MethodsWe retrospectively investigated pemphigus patients with a history of clinical remission who visited the Department of Dermatology of Keio University during 2019 and 2020. The antibody pathogenicity was assessed by bead aggregation assay.ResultsWhen patients were recognized as having entered remission (PDAI = 0 and PSL ≦ 10 mg/day for 2 months), serum autoantibodies against Dsg were detected in 72 of 132 patients (54.5%, positive group; PG), but were not detected in 60 patients (45.5%, negative group; NG). Anti‐Dsg antibody titres in remission declined from the active phase in 33 patients in the PG for whom data were available. There were no differences in the chance of reducing PSL to 5 mg/day (P= 0.885) and rate of relapse (P= 0.279) between PG and NG, but fewer patients in PG discontinued corticosteroids (P= 0.004). The ability of patients' sera to block aggregation of Dsg/desmocollin beads was significantly reduced in remission compared to the active phase. However, our results revealed that whole sera in remission still had pathogenic activity in seven of nine patients, and the approximately equal amounts of anti‐Dsg antibodies in active phase and remission showed similar pathogenicity.ConclusionsThis study will provide guidance in cases where autoantibodies are found to be positive in pemphigus patients during remission or steroid reduction.