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免疫球蛋白G4相關疾病一例

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 相關性淋巴結病一例

免疫球蛋白G4相關性甲狀腺疾病

免疫球蛋白 G4 相關性疾病（immunoglobulin G4-related disease，IgG4-RD）是本世紀初新認識的可累及包括內分泌系統特別是甲狀腺的全身多器官系統疾病，以免疫介導的纖維化炎癥為主要病理特征。免疫球蛋白 G4 相關甲狀腺疾病（immunoglobulin G4-related thyroid disease，IgG4-RTD）是很少被考慮到的一類甲狀腺疾病，既可單獨累及甲狀腺也可同時累及其他器官。目前認為 IgG4-RTD 包括 4 種亞型：Riedel 甲狀腺炎、橋本甲狀腺炎的纖維樣變型、免疫球蛋白 G4（immunoglobulin G4，IgG4）相關性橋本甲狀腺炎和 Graves 病合并 IgG4 升高。其診斷較為復雜，需結合臨床表現、組織學特征和血清學證據綜合判斷。大多數情況下，可根據經典的組織病理學表現診斷 IgG4-RTD，因此強烈推薦在治療前進行活檢。IgG4-RTD 的治療包括藥物治療和手術治療，雖然可能需要進一步證據，類固醇仍是一線治療藥物。他莫昔芬和利妥昔單抗是類固醇抵抗患者的二線治療藥物。對于有壓迫癥狀患者應選擇行甲狀腺切除。到目前為止，該病的病理生理機制尚未完全明確，早期及時診斷、早期治療可明顯改善預后。