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IgG4相关性疾病患者内分泌腺体受累的临床特征分析

Clinical characteristics of endocrine glands involvement in patients with IgG4-related diseases
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摘要 目的 探讨IgG4相关性疾病(IgG4-RD)患者内分泌腺体受累的临床表现、影像学与病理学特征、治疗及疾病转归。方法对2014年1月1日至2018年6月30日北京协和医院收治的10例IgG4-RD伴内分泌腺体受累患者进行临床资料收集和分析,并总结其临床特点。结果 在10例IgG4-RD患者中,男性4例,女性6例,中位发病年龄为55岁。单一器官受累者5例(仅垂体受累1例.甲状腺受累4例),≥2个器官受累者5例。8例患者C反应蛋白或红细胞沉降率升高,7例抗核抗体阳性,6例血清总IgE升高。9例患者予以口服糖皮质激素治疗,其中3例联合免疫抑制剂或美罗华治疗,1例随诊观察。患者临床症状均有缓解,血清IgG水平均较前明显下降,5例患者血清IgG降至正常范围。同时影像学较前改善。结论对于多个内分泌腺体受累并伴IgG4升高的患者,均需考虑IgG4-RD的可能。必要时行组织活检病理检查。 Objective To explore the clinical manifestations, imaging and pathology features, treatment, and prognosis of endocrine glands involved patients with IgG4-related disease ( IgG4-RD ). Methods Ten patients admitted in Peking Union Medical College Hospital from 1 January 2014 to 30 June 2018 diagnosed as IgG4-RD with endocrine glands involved were enrolled in this study. All the clinical data were collected and analyzed. Results Ten patients, 4 males and 6 females, median 55 years old at the onset were enrolled, five patients with single organ involvement ( 1 case involved in pituitary, and 4 cases involved in thyroid) , while another 5 patients with 2 and more organs involved. C-reactive protein or erythrocyte sedimentation rate was elevated in 8 patients, antinuclear antibodies were positive in 7, and serum total IgE was elevated in 6 patients. Nine patients were treated with oral glucoeorticoids, among whom 3 patients were treated in combination with immunosuppressive agents or rituximab. After treatment, the clinical symptoms were alleviated and imaging was improved in all patients. Serum IgG levels were significantly decreased in all patients and normalized in 5 patients. Conclusion For the patients with multiple endocrine gland diseases and elevated IgG4 level, the possibility of IgG4-RD should be carefully considered and should be confirmed by tissue biopsy and histopathology.
作者 吴晗 于淼 童安莉 冯凯 许岭翎 胡明明 段炼 刘巍 邢小平 Wu Han,Yu Miao,Tong Anli,Feng Kai,Xu Lingling,Ha Mingming,Duan Lian,Liu Wei,Xing Xiaoping(Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China)
出处 《中华内分泌代谢杂志》 CSCD 北大核心 2018年第10期839-843,共5页 Chinese Journal of Endocrinology and Metabolism
基金 中国医学科学院医学与健康科技创新工程基金(CIFMS2017-12M-1-008)
关键词 lgG4相关性疾病 垂体 甲状腺 胰腺 IgG4-related disease Pituitary Thyroid Pancreas
作者简介 通信作者:于淼,Email:yumiaoxh@163.com
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