Analysis of blistering mechanism in pemphigus.

天疱疮是一种难治性自身免疫性水疱性疾病，由针对桥粒芯糖蛋白（Dsgs）胞外结构域（EC）的IgG自身抗体引起。我们已经修改了酶联免疫吸附试验（ELISA），使用乙二胺四乙酸（EDTA）检测非致病性抗Dsg3抗体对非钙（Ca2+）依赖性表位。使用该方法，可以获得针对Ca 2+依赖性表位的抗Dsg3血清抗体。在这项研究中，我们检查了ELISA测定的抗Dsg3血清抗体对钙依赖性表位的活性在临床病例中的可靠性，并证明了ELISA测定的抗Dsg3血清抗体对钙依赖性构象表位的活性和PV患者的临床疾病活动之间的显着相关性。构象Dsg3 ELISA指数显示改善的相关性，疾病的活动时，在个别情况下进行监测。

Pemphigus is an intractable autoimmune blistering disease caused by IgG autoantibodies against the extracellular domains (ECs) of desmogleins (Dsgs). We have modified enzyme-linked immunosorbent assay (ELISA) using ethylenediaminetetraacetic acid (EDTA) to detect only nonpathogenic anti-Dsg3 antibodies against the non-calcium (Ca2+)-dependent epitopes. Using this method, anti-Dsg3 serum antibodies against the Ca2+-dependent epitopes can be obtained. In this study, we examined the reliability of the ELISA-determined activities of anti-Dsg3 serum antibodies against the Ca2+-dependent epitopes in clinical cases and demonstrated a significant correlation between the ELISA-determined activities of anti-Dsg3 serum antibodies against the Ca2+-dependent conformational epitopes and the clinical disease activities of PV patients. The conformational Dsg3 ELISA index showed improved correlation to the disease activity when monitored in individual cases.