Table_1_Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid.xlsx

Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. With the exception of one study, pointing toward a decrease of IgG in pemphigus patients, with a relative enrichment of IgG4, serum immunoglobulin (Ig) concentrations had not been studied in pemphigus and pemphigoid. Hence, we here aimed to investigate serum concentrations of IgM, IgA, IgG, and IgG1–4 in pemphigus and pemphigoid patients, as well as in healthy controls. Serum Ig concentrations were determined by ELISA in 105 healthy controls, 100 pemphigus vulgaris (PV), 100 pemphigus foliaceus, 99 bullous pemphigoid (BP), and 55 linear IgA bullous dermatosis (LAD) patients. In healthy controls, age had a significant impact on Ig serum concentrations: In controls at ages of 69 years or older, IgM and IgG were decreased, while all other Ig, except IgA and IgG4, were increased. When stratified by sex, lower IgM concentrations were observed in males. When corrected for age and/or sex, and compared to controls, an increase in serum IgA was noted in LAD. In almost all patient cohorts, an increase in IgG1 and IgG4 was observed, while a decrease in IgG2 or IgG3 was seen in BP or PV patients. This points toward a possible association of BP with IgG2 deficiency and warrants evaluation of IgG2 in BP patients prior to immunosuppressive therapy.

天疱疮及其类天疱疮疾病为皮肤及/或粘膜的器官特异性自身免疫性疾病，其由针对皮肤结构蛋白的自身抗体引起。在其他自身免疫性疾病中，观察到原发性抗体缺乏症的普遍存在。反之，在原发性抗体缺乏症患者中，也有报道称其自身免疫性疾病的高发率。除了一项研究指出天疱疮患者中IgG减少，而IgG4相对富集外，血清免疫球蛋白（Ig）浓度在天疱疮及其类天疱疮疾病中尚未得到研究。因此，本研究旨在调查天疱疮及其类天疱疮患者，以及健康对照者的血清IgM、IgA、IgG和IgG1–4浓度。血清Ig浓度通过ELISA在105名健康对照者、100名寻常型天疱疮（PV）、100名落叶型天疱疮、99名大疱性类天疱疮（BP）和55名线性IgA大疱性皮炎（LAD）患者中测定。在健康对照者中，年龄对Ig血清浓度有显著影响：在69岁或以上的对照组中，IgM和IgG减少，而除IgA和IgG4外的所有其他Ig浓度均升高。按性别分层时，观察到男性中IgM浓度较低。在调整年龄和/或性别后，与对照者相比，LAD患者血清IgA浓度增加。在几乎所有的患者群体中，观察到IgG1和IgG4的增加，而在BP或PV患者中观察到IgG2或IgG3的减少。这表明BP可能与IgG2缺乏有关，并需要在免疫抑制治疗前评估BP患者的IgG2浓度。