Bhol K, Mohimen A, Ahmed A R
Department of Dermatology, Boston University School of Medicine, Mass 02118.
Dermatology. 1994;189 Suppl 1:85-9. doi: 10.1159/000246938.
Pemphigus vulgaris is an autoimmune blistering disease that is characterized by the presence of an antibody against an epidermal cell protein. In our previous studies, we have demonstrated that the presence of the antibody in the patients and in the healthy relatives of the patients strongly correlates with MHC haplotypes. The purpose of this study was to determine the subclasses of IgG present in the sera of patients with active disease, those in remission, healthy and unaffected relatives and normal controls. The presence of the autoantibody to the pemphigus antigen was assayed by a modified, sensitive immunoblot technique. The assay was considered positive if a 130-kD band was seen when normal human epidermis was used as a substrate. In patients with active disease, the sera contained antibodies of the IgG1 and IgG4 subclasses. The sera of patients in remission, those of healthy unaffected relatives and normal controls contained only the IgG1 subclass. The normal controls consist of MHC-matched and nonmatched individuals. This study indicates that patients with disease activity have an antibody which is of the IgG1 and IgG4 subclasses, which we consider to be a pathogenic antibody. The sera of healthy relatives and normal controls that contain an antibody which binds to the pemphigus antigen is of the IgG1 subclass only and is considered to be a nonpathogenic or natural autoantibody. These observations provide the basis to study the immunoregulatory mechanisms and the production of normal and pathogenic antibodies.
寻常型天疱疮是一种自身免疫性水疱病，其特征是存在针对表皮细胞蛋白的抗体。在我们之前的研究中，我们已经证明患者及其健康亲属体内抗体的存在与MHC单倍型密切相关。本研究的目的是确定活动期患者、缓解期患者、健康未患病亲属以及正常对照血清中存在的IgG亚类。采用改良的敏感免疫印迹技术检测针对天疱疮抗原的自身抗体的存在。以正常人表皮为底物时，若出现130-kD条带，则该检测被认为呈阳性。活动期患者的血清中含有IgG1和IgG4亚类抗体。缓解期患者、健康未患病亲属以及正常对照的血清中仅含有IgG1亚类。正常对照由MHC匹配和不匹配的个体组成。本研究表明，处于疾病活动期的患者具有IgG1和IgG4亚类抗体，我们认为这是一种致病抗体。健康亲属和正常对照的血清中与天疱疮抗原结合的抗体仅为IgG1亚类，被认为是非致病或天然自身抗体。这些观察结果为研究免疫调节机制以及正常和致病抗体的产生提供了基础。
