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免疫球蛋白G4相关疾病一例

Serum Immunoglobulin G4 Level in 32 Patients with Active-phase Pemphigus Vulgaris

ObjectiveTo learn the serum immunoglobulin G4 (IgG4) level in patients with pemphigus vulgaris (PV) in the active phase by detecting the IgG4 concentration in the patients with PV. MethodsWe collected the serum samples from patients with active-phase PV (including acute-onset and chronic active phases) between April and December 2014. The serum IgG4 level was detected by immuno-scatter turbidmetry, and the difference of serum IgG4 level between PV patients and normal subjects was assessed by Student's t-test. ResultsThirty-two patients with pemphigus vulgaris in the active phase were enrolled for this study, among whom 22 (including 8 males and 14 females) had normal serum IgG4 level, 8 (including 3 males and 5 females) higher and 2 (including 1 male and 1 female) lower. Compared with normal subjects, the average level of serum IgG4 in PV patients was not significantly different (P>0.05). The serum IgG4 level of acute-onset and chronic active PV patients was (1.015±0.825) g/L and (1.058±1.133) g/L, respectively, with no significant difference (P>0.05). ConclusionThe serum IgG4 level of patients with pemphigus vulgaris in the active phase can be normal, higher or lower. And there is no obvious difference in the serum IgG4 level between acute-onset and chronic active PV patients.

免疫球蛋白 G4 相关性淋巴结病一例

免疫球蛋白G4相关性甲状腺疾病

免疫球蛋白 G4 相关性疾病（immunoglobulin G4-related disease，IgG4-RD）是本世纪初新认识的可累及包括内分泌系统特别是甲状腺的全身多器官系统疾病，以免疫介导的纤维化炎症为主要病理特征。免疫球蛋白 G4 相关甲状腺疾病（immunoglobulin G4-related thyroid disease，IgG4-RTD）是很少被考虑到的一类甲状腺疾病，既可单独累及甲状腺也可同时累及其他器官。目前认为 IgG4-RTD 包括 4 种亚型：Riedel 甲状腺炎、桥本甲状腺炎的纤维样变型、免疫球蛋白 G4（immunoglobulin G4，IgG4）相关性桥本甲状腺炎和 Graves 病合并 IgG4 升高。其诊断较为复杂，需结合临床表现、组织学特征和血清学证据综合判断。大多数情况下，可根据经典的组织病理学表现诊断 IgG4-RTD，因此强烈推荐在治疗前进行活检。IgG4-RTD 的治疗包括药物治疗和手术治疗，虽然可能需要进一步证据，类固醇仍是一线治疗药物。他莫昔芬和利妥昔单抗是类固醇抵抗患者的二线治疗药物。对于有压迫症状患者应选择行甲状腺切除。到目前为止，该病的病理生理机制尚未完全明确，早期及时诊断、早期治疗可明显改善预后。