Analysis of blistering mechanism in pemphigus.

天疱疮是一种难治性自身免疫性起疱性疾病，由IgG自身抗体对抗桥粒蛋白（Dsgs）的细胞外结构域（ECs）引起。我们使用乙二胺四乙酸（EDTA）改进了酶联免疫吸附试验（ELISA），仅检测针对非钙（Ca2+）依赖性表位的非致病性抗dsg3抗体。使用这种方法，可以获得针对Ca2+依赖性表位的抗dsg3血清抗体。在这项研究中，我们检验了临床病例中抗dsg3血清抗体抗Ca2+依赖表位活性的可靠性，并证明了elisa测定的抗dsg3血清抗体抗Ca2+依赖构象表位活性与PV患者临床疾病活动之间存在显著相关性。在个别病例监测时，构象Dsg3 ELISA指数与疾病活动性的相关性有所提高。

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.