Objective: To analyze the clinical and pathological features of Cronkhite-Canada syndrome (CCS), and to investigate the significance of IgG4 positive plasma cell infiltration. Methods: Clinical presentations, endoscopic appearances and morphological features of 18 patients diagnosed with CCS at Peking Union Medical College Hospital during 2000-2016 were included in the study.There were 11 male and 7 female patients. IgG4 and IgG immunohistochemical stains were performed in total of 55 biopsies from the patients (36 polyps, 10 adenoma and 9 surrounding mucosa) and a control group of 58 cases (19 colonic mucosa, 7 colonic hyperplastic polyps, 9 inflammatory bowel disease and 23 adenoma). Results: Average age of CCS was 59 years (range 47-69 years) and the male to female ratio was 11∶7. All patients had at least one ectoderm lesion. Fourteen cases had testicular abnormalities. Digestive tract symptoms were encountered in most cases. Four patients had a history of connective tissue disease. Endoscopically, multiple polyps were found to involve entire gastrointestinal tract except the esophagus. Morphologically, CCS polyps were characterized by prominent mucosal edema, mild to moderate inflammation, glands hyperplasia and cystic dilatation. Ten cases had colonic adenoma and one case had rectal adenocarcinoma. While none of the cases reached the diagnostic criteria of IgG4 disease, the positive rate of IgG4 positive plasma cells in adenoma of CCS was significantly higher than that in polyps and surrounding mucosa of CCS (60.0%∶13.9%∶0, P<0.01). The positive rate of IgG4 positive plasma cells of adenoma and polyp of CCS had an increased tendency compared to that of adenoma and polyp of control group (60.0%∶34.8%; 13.9%∶0). Conclusion: The findings in the study do not support that CCS is a variant of IgG4 disease, although IgG4 positive plasma cells may be involved in the pathogenesis of CCS, which may be explored in future investigations.
目的： 分析Cronkhite－Canada综合征(CCS)的临床病理学特征，并探讨IgG4阳性浆细胞浸润的意义。 方法： 纳入北京协和医院2000至2016年确诊的18例CCS患者，男性11例，女性7例。总结临床症状、内镜及形态学特征。对18例患者共55份活检标本(CCS息肉36份，腺瘤10份，息肉周围黏膜组织9份)和对照组58例(正常结肠黏膜19例，结肠增生性息肉7例，炎症性肠病9例，结肠管状腺瘤23例)进行IgG4和IgG免疫组织化学染色。 结果： CCS患者确诊年龄平均为59岁(47～69岁)，男女比11∶7。所有患者均有至少1项外胚层病变，14例患者伴有味觉异常，常伴消化道症状，4例患者伴结缔组织病史。内镜以除食管外的全消化道多发息肉为特点，胃及结肠最易受累。镜下CCS息肉为错构瘤性，固有膜高度水肿，轻－中度炎性细胞浸润，腺体增生伴囊性扩张，病变可累及息肉间黏膜。10例患者伴结肠管状腺瘤，1例患者合并直肠腺癌。所有CCS患者均未达到IgG4相关疾病的病理确诊标准。CCS腺瘤中IgG4阳性浆细胞阳性率明显高于CCS息肉及CCS周围黏膜(60.0%比13.9%比0，P<0.01)。CCS腺瘤以及息肉中IgG4阳性细胞阳性率具有高于对照组的趋势(60.0%比34.8%；13.9%比0)。 结论： 不支持CCS是一类IgG4相关疾病的观点。但IgG4阳性浆细胞可能参与CCS的病理生理变化过程，其具体作用及机制有待进一步探讨。.
Keywords: Autoimmune diseases; Cronkhite-Canada syndrome; Intestinal polyposis; Plasma cells.