Lee Sang Hee, Hong Won Jin, Kim Soo-Chan
Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Ann Dermatol. 2017 Aug;29(4):438-445. doi: 10.5021/ad.2017.29.4.438. Epub 2017 Jun 21.
Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized.
This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP) and healthy subjects.
A total of 75 subjects (28 PV, 13 PF, 7 PNP, 7 BP, and 20 healthy controls) were included, all patients in active disease state. Serum levels of interferon (IFN)-γ, interleukin (IL)-4, IL-6, IL-17A, IL-10, tumor necrosis factor (TNF)-α, and IL-8 were measured by enzyme-linked immunosorbent assay.
The median concentration of IFN-γ was lower in PV and BP patients compared to control (0.77, 0.34 and 1.63 pg/ml, respectively). IL-6 and IL-10 was significantly higher in PNP patients compared to control (4.92 and 0.24 pg/ml for IL-6, 0.86 and <0.12 pg/ml for IL-10, respectively). IL-8 was increased significantly in PV and PNP patients compared with control (11.85, 31.5 and 8.31 pg/ml, respectively). For IL-4, IL-17A and TNF-α, no significant difference was observed between the five groups.
The decreased level of IFN-γ in PV may imply suppressed Th1 response in the active disease stage. A Th2 predominant response is suggested in the active stage of PNP, with elevated serum levels of IL-6 and IL-10. Increased level of proinflammatory cytokine IL-8 is observed in the sera of PV and PNP patients.
天疱疮是一组影响皮肤和黏膜的自身免疫性水疱性疾病。虽然天疱疮是一种自身抗体介导的疾病，但T细胞和细胞因子在发病机制中的作用正日益受到认可。
本研究旨在观察寻常型天疱疮（PV）、落叶型天疱疮（PF）、副肿瘤性天疱疮（PNP）患者血清细胞因子水平的变化，并与大疱性类天疱疮（BP）和健康受试者进行比较。
共纳入75名受试者（28例PV、13例PF、7例PNP、7例BP和20名健康对照），所有患者均处于疾病活动期。采用酶联免疫吸附测定法检测血清干扰素（IFN）-γ、白细胞介素（IL）-4、IL-6、IL-17A、IL-10、肿瘤坏死因子（TNF）-α和IL-8水平。
与对照组相比，PV和BP患者的IFN-γ中位浓度较低（分别为0.77、0.34和1.63 pg/ml）。与对照组相比，PNP患者的IL-6和IL-10显著升高（IL-6分别为4.92和0.24 pg/ml，IL-10分别为0.86和<0.12 pg/ml）。与对照组相比，PV和PNP患者的IL-8显著升高（分别为11.85、31.5和8.31 pg/ml）。对于IL-4、IL-17A和TNF-α，五组之间未观察到显著差异。
PV中IFN-γ水平降低可能意味着在疾病活动期Th1反应受到抑制。PNP活动期提示以Th2为主的反应，血清IL-6和IL-10水平升高。在PV和PNP患者的血清中观察到促炎细胞因子IL-8水平升高。
