Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus
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DOI：
10.1111/j.1365-2133.2006.07717.x
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发表时间：
2007-04-01
影响因子：
10.3
通讯作者：
Pas, H. H.
中科院分区：
文献类型：
--
作者：
Mentink, L. F.;de Jong, M. C. J. M.;Pas, H. H.
天疱疮是一种大疱性粘膜皮肤自身免疫性疾病，其特征是IgG自身抗体对抗粘粒蛋白（Dsg） 1和/或Dsg3。有时天疱疮皮肤的直接免疫荧光显示除了IgG沉积外，还有表皮内细胞间模式的IgA沉积。目的探讨天疱疮患者除IgG自身抗体外，是否还产生针对Dsg1和/或Dsg3的IgA抗体。方法采用IgA酶联免疫吸附试验（ELISA）检测100例天疱疮患者和36例大疱性类天疱疮对照组血清中Dsg1和Dsg3重组胞外结构域的表达。根据临床情况和Dsg1和/或Dsg3的IgG ELISA指数阳性选择患者。他们被分为四组：IgG仅对Dsg1 (n = 34)， IgG同时对Dsg1和Dsg3 (n = 31)， IgG仅对Dsg3 （n = 27）和副肿瘤天疱疮（PNP）患者（n = 8）。结果Dsg1抗体IgG患者中有13例（38%）存在Dsg1抗体，Dsg1和Dsg3抗体IgG患者中有5例（16%）存在，Dsg3抗体IgG患者中有4例（15%）存在，PNP患者中无IgA抗体。在Dsg1抗体IgG患者中有1例（3%）出现Dsg3抗体，在Dsg1和ds3抗体IgG患者中有18例（58%）出现，在Dsg3抗体IgG患者中有18例（67%）出现，在PNP患者中有4例（50%）出现。免疫荧光分析显示，IgA ELISA阳性患者血清中存在表皮细胞间染色IgA抗体，皮肤中存在细胞间IgA沉积，但程度低于ELISA。结论：本研究表明，在相当一部分被认为是igg介导的天疱疮患者中，也存在对Dsg1和Dsg3的IgA。在大多数情况下，IgA的抗原特异性跟随IgG的抗原特异性，尽管在少数情况下IgA对IgG不能识别的Dsg存在。
Background Pemphigus is a bullous mucocutaneous autoimmune disease characterized by IgG autoantibodies to desmoglein (Dsg) 1 and/or Dsg3. Occasionally direct immunofluorescence of pemphigus skin reveals IgA depositions with an intraepidermal intercellular pattern in addition to the IgG deposition.Objectives To investigate if pemphigus patients, in addition to having IgG autoantibodies, also generate IgA antibodies to Dsg1 and/or Dsg3.Patients/methods Sera of 100 pemphigus patients and 36 bullous pemphigoid controls were tested by IgA enzyme-linked immunosorbent assay (ELISA) to the recombinant extracellular domains of Dsg1 and Dsg3. The patients were selected on clinical grounds and positive IgG ELISA index values for Dsg1 and/or Dsg3. They were divided into four groups: patients having IgG to only Dsg1 (n = 34), patients having IgG to both Dsg1 and Dsg3 (n = 31), patients having IgG to only Dsg3 (n = 27) and patients who had paraneoplastic pemphigus (PNP) (n = 8).Results IgA antibodies to Dsg1 were found in 13 (38%) of the patients with IgG to Dsg1, in five (16%) of the patients with IgG to both Dsg1 and Dsg3, in four (15%) of the patients with IgG to Dsg3 and in none of the PNP patients. IgA antibodies to Dsg3 were found in one (3%) of the patients with IgG to Dsg1, in 18 (58%) of the patients with IgG to both Dsg1 and 3, in 18 (67%) of the patients with IgG to Dsg3, and in four (50%) of the PNP patients. Immunofluorescence analysis demonstrated intraepidermal intercellular staining IgA antibodies in serum and intercellular IgA deposits in skin of IgA ELISA-positive patients, although to a lesser extent than by ELISA.Conclusions This study shows that in a considerable number of supposedly IgG-mediated pemphigus patients IgA to Dsg1 and Dsg3 is also present. In most cases the antigen specificity of the IgA follows the antigen specificity of the IgG, although in a small number of cases IgA is present against the Dsg not recognized by IgG.