The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels
The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels
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DOI：
10.1046/j.1365-2133.2001.04132.x
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发表时间：
2001-04-01
影响因子：
10.3
通讯作者：
Black, MM
中科院分区：
文献类型：
--
作者：
Harman, KE;Seed, PT;Black, MM
背景 寻常型天疱疮 (PV) 和落叶型天疱疮 (PF) 的特征是分别针对桥粒蛋白桥粒芯糖蛋白 3 (Dsg3) 和桥粒芯糖蛋白 1 (Dsg1) 的抗体。过去使用间接免疫荧光 (IIF) 作为天疱疮抗体水平测量指标的研究未能一致证明疾病严重程度与 IIF 滴度之间的关系。然而，与利用重组蛋白的酶联免疫吸附测定 (ELISA) 不同，IIF 无法单独测量 Dsg1 和 3 抗体。 目的 独立比较 Dsg1 和 3 抗体水平与 PV 和 PEP 中皮肤和口腔受累的严重程度 患者和方法 分析了 80 名 PV 受试者和 24 名 PE 受试者的 424 份血清样本 通过 ELISA 测量了 Dsg1 和 3 的 IgG 抗体，对于每个样本经分析，皮肤和口腔疾病的相关严重程度分为0至3级；结果 Dsg1 抗体与皮肤严重程度之间存在关系，Dsg1 ELISA 值每增加 10 个单位，就有 34% 的机会获得更高的严重程度评分 [95% 置信区间 (CI)，25-45%，P < 0.0005]。在 PV 和 PE 中均观察到这一点。口腔严重程度与 Dsg3 抗体水平相关，Dsg3 ELISA 值增加 10 个单位与口腔严重程度评分较高的 25% 机会相关（CT 17-33%，P < 0.0005）。即使在调整了 Dsg3 抗体的影响后，我们也无法证明 Dsg1 抗体与口腔严重程度之间的关系，同样，Dsg3 抗体与皮肤严重程度之间也没有关系。结论这项研究表明，天疱疮的临床表型，特别是皮肤和口腔疾病的平衡，主要分别由 Dsg1 和 3 自身抗体的数量决定。
Background Pemphigus vulgaris (PV) and foliaceus (PF) are characterized by antibodies to the desmosomal proteins desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1), respectively. Past studies using indirect immunofluorescence (IIF) as a measure of pemphigus antibody levels have failed to demonstrate consistently a relationship between disease severity and IIF titres. However, IIF is not able to measure separately Dsg1 and 3 antibodies, unlike enzyme-linked immunosorbent assays (ELISA), which utilize recombinant proteins.Objectives To compare independently Dsg1 and 3 antibody levels with the severity of both cutaneous and oral involvement in PV and PEPatients and methods Four hundred and twenty-four serum samples were analysed from 80 subjects with PV and 24 with PE IgG antibodies to Dsg1 and 3 were measured by ELISA, For every sample analysed, the associated severity of skin and oral disease were graded from 0 to 3; quiescent, mild, moderate and severe.Results A relationship between Dsg1 antibodies and skin severity was demonstrated such that a 10-unit increase in Dsg1 ELISA value was associated with a 34% chance of having a higher severity score [95% confidence interval (CI), 25-45%, P < 0.0005]. This was observed in both PV and PE Oral severity was associated with Dsg3 antibody levels and a 10-unit increase in the Dsg3 ELISA value was associated with a 25% chance of a higher oral severity score (CT 17-33%, P < 0.0005). We were unable to demonstrate a relationship between Dsg1 antibodies and oral severity, even after adjusting for the effect of Dsg3 antibodies, Similarly, there was no relationship between Dsg3 antibodies and skin severity,Conclusions This study suggests that the clinical phenotype of pemphigus, in particular the balance of skin and oral disease, is determined principally by the quantities of Dsg1 and 3 autoantibodies, respectively.