Cronkhite-Canada综合征的血清免疫球蛋白特征:单中心回顾性研究

Characteristics of Serum Immunoglobulin in Cronkhite-Canada Syndrome: A Single Center Retrospective Study

  • 摘要:
    目的 探讨Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)患者血清免疫球蛋白特征及其临床价值。
    方法 纳入2009年12月至2024年9月于北京协和医院收治并进行血清免疫球蛋白检测的CCS患者,回顾性分析其临床特点及辅助检查结果。
    结果 共纳入52例患者,男36例(69.2%),女16例(30.8%),中位起病年龄60(54~64)岁,起病至诊断的中位时间为5(3~12)个月。常见临床表现包括腹泻(86.5%)、腹痛(40.4%)、便血(19.2%)、体重下降(86.5%)、指甲营养不良(100.0%)、色素沉着(88.5%)、毛发脱落(84.6%)、味觉减退(69.2%)等。21例(50.0%)患者存在血清IgG下降,20例(71.4%)的患者存在总IgE升高。11例(23.4%)血清IgG4高于参考值,IgG4升高组与IgG4正常组间的临床指标无显著差异,活动期CCS患者(n=43)与缓解期患者(n=27)的IgG4水平比较差异无统计学意义。总IgE升高组的毛发脱落率显著高于IgE正常组(P=0.0383)。
    结论 CCS患者存在外周血免疫球蛋白紊乱,其中IgE升高与毛发脱落有关,提示CCS患者的毛发脱落可能与免疫介导机制相关。

     

    Abstract:
    Objective To investigate the characteristics and clinical significance of serum immunoglobulins in patients with Cronkhite-Canada syndrome (CCS).
    Methods This retrospective study included CCS patients admitted to Peking Union Medical College Hospital from December 2009 to September 2024 who underwent serum immunoglobulin testing. Clinical manifestations and ancillary examination results were analyzed retrospectively.
    Results Fifty-two patients were included (male: 3669.2%, female: 1630.8%), with median onset age of 60 (54-64) years and median diagnostic delay of 5 (3-12) months. Common manifestations included diarrhea (86.5%), abdominal pain (40.4%), hematochezia (19.2%), weight loss (86.5%), nail malnutrition (100.0%), pigmentation (88.5%), hair loss (84.6%), and hypogeusia (69.2%). Serum IgG reduction occurred in 21 patients (50.0%), while total IgE elevation was observed in 20(71.4%). Elevated serum IgG4 levels were noted in 11 patients (23.4%), with no significant clinical differences between IgG4-elevated and normal groups. IgG4 levels showed no statistical difference between active (n=43) and remission (n=27) groups. The hair loss rate was significantly higher in the total IgE-elevated group than that in the normal total IgE group (P=0.0383).
    Conclusions CCS patients exhibit peripheral blood immunoglobulin disorders, with elevated IgE levels correlating with hair loss. This suggests an immune-mediated mechanism may underlie hair loss in CCS.

     

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