常见疾病的罕见表现:中年男性多尿-头痛-炎症肉芽肿性病变  

Rare Manifestations of Common Diseases:Middle-Aged Male Polyuria-Headache-Inflammatory Granulomatous Lesions

作　　者：赵宇星 段炼 吕玮[2] 姚勇[3] 有慧[4] 张文[5] 马瑾[6] 毛歆歆[7] 朱惠娟 ZHAO Yuxing;DUAN Lian;LYU Wei;YAO Yong;YOU Hui;ZHANG Wen;MA Jin;MAO Xinxin;ZHU Huijuan(Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Infectious Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Neurosurgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Radiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Rheumatology and Immunology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Ophthalmology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院内分泌科、国家卫生健康委员会内分泌重点实验室,北京100730 [2]中国医学科学院北京协和医院感染科,北京100730 [3]中国医学科学院北京协和医院神经外科,北京100730 [4]中国医学科学院北京协和医院放射科,北京100730 [5]中国医学科学院北京协和医院风湿免疫科,北京100730 [6]中国医学科学院北京协和医院眼科,北京100730 [7]中国医学科学院北京协和医院病理科,北京100730

出　　处：《罕见病研究》2023年第3期359-364,共6页Journal of Rare Diseases

基　　金：中央高水平医院临床科研业务费(2022-PUMCH-B-016)。

摘　　要：中年男性以纳差、多尿、多饮、头痛起病,发现鞍区占位性病变伴全垂体功能减退、视野缺损。经鼻蝶鞍区占位活检,病理提示炎症改变伴肉芽肿形成,多次脑脊液及病理组织病原学检查无阳性发现,诊断为自身免疫性垂体炎,给予糖皮质激素联合免疫抑制剂治疗,鞍区病灶有一过性缩小后较前逐步增大。经多学科病例讨论后考虑垂体结核感染可能性大,给予规范抗结核治疗后,病灶较前明显缩小,病情改善。垂体结核感染非常罕见,诊断困难,极易误诊,该病例通过多学科诊疗得到及时有效的诊治,体现了罕见病多学科诊疗的重要意义。A middle-aged man was presented with poor appetite,polyuria,polydrpsia,and headache.A sellar mass was found,along with total pituitary hypofunction and visual field defect.A biopsy of the lesion via the trans-sphenoidal approach showed inflammatory changes and granuloma formation.However,repeated cerebrospinal fluid and pathogenic examination of the pathological tissue showed no positive indications.The initial diagnosis considered autoimmune hypophysitis,and treatment of glucocorticoids combined with immunosuppressants was administered,which led to a temporary shrinkage of the lesion,but it gradually enlarged subsequently.After multidisciplinary discussion,a high possibility of pituitary tuberculosis infection was decided upon.After standardized anti-tuberculosis treatment was initiated,the lesion reduced noticeably and the patient's condition improved.Pituitary tuberculosis infection is incredibly rare and extremely easy to misdiagnose.This case was diagnosed and treated in a timely and effective manner through a multidisciplinary approach,highlighting the importance of such an approach in dealing with rare diseases.

关 键 词：垂体结核 鞍区占位 全垂体功能减退 垂体炎 

分 类 号：R529.3[医药卫生—内科学] R584.2[医药卫生—临床医学]