Immunology Department, La Rabta Hospital, Sfax, Tunisia.
J Eur Acad Dermatol Venereol. 2011 Jul;25(7):774-81. doi: 10.1111/j.1468-3083.2010.03859.x. Epub 2010 Oct 3.
Pemphigus is a life-threatening autoimmune blistering disease mediated by autoantibodies against adhesion molecule of the skin. Its concurrence with systemic and organ-specific autoimmune disease was described in case reports.
To evaluate the presence of a broad spectrum of organ-specific and non-organ-specific autoantibodies other than anti-desmoglein antibodies in pemphigus patients.
Serum samples were obtained from 105 pemphigus foliaceus (PF) patients, 51 pemphigus vulgaris (PV) patients and 50 controls. Both indirect immunofluorescence assay and ELISA were used to assess the presence of autoantibodies related to connective tissue diseases, autoimmune hepatitis, vasculitis, rheumatoid arthritis, coeliac disease, diabetes and thyroiditis.
Significant difference was observed between the three groups for anti-thyroglobulin antibodies in the pemphigus foliaceus group (18% vs. 4%, P=0.03). A significantly higher occurrence of IgM anti-cardiolipin (P=0.03), IgG anti-reticulin (P=0.01) and IgG anti-gliadin antibodies (P=0.008) were observed in the PV group. Cases with more than four autoantibodies were frequently positives for both anti-desmoglein 1 and anti-desmoglein 3.
Autoantibodies other than anti-desmoglein antibodies are not rare in pemphigus patients. Clinical and serological follow-up of pemphigus patients with positive autoantibodies are needed to clarify their impact in disease evolution.
天疱疮是一种危及生命的自身免疫性水疱病,由针对皮肤黏附分子的自身抗体介导。其与系统性和器官特异性自身免疫性疾病的并发在病例报告中有所描述。
评估天疱疮患者除抗桥粒芯糖蛋白抗体以外,是否存在广泛的器官特异性和非器官特异性自身抗体。
采集 105 例天疱疮患者(PF 组)、51 例寻常型天疱疮患者(PV 组)和 50 例对照者的血清样本。采用间接免疫荧光法和酶联免疫吸附试验评估与结缔组织疾病、自身免疫性肝炎、血管炎、类风湿关节炎、乳糜泻、糖尿病和甲状腺炎相关的自身抗体。
在 PF 组中,抗甲状腺球蛋白抗体在天疱疮患者组和对照组之间存在显著差异(18%比 4%,P=0.03)。在 PV 组中,IgM 抗心磷脂(P=0.03)、IgG 抗网状蛋白(P=0.01)和 IgG 抗麦胶蛋白抗体(P=0.008)的发生率明显更高。出现超过四种自身抗体的病例通常抗桥粒芯糖蛋白 1 和抗桥粒芯糖蛋白 3 均为阳性。
天疱疮患者中并非罕见存在除抗桥粒芯糖蛋白抗体以外的自身抗体。需要对自身抗体阳性的天疱疮患者进行临床和血清学随访,以阐明其对疾病进展的影响。