自身免疫性垂体炎的诊治进展  

Progress in Diagnosis and Treatment of Autoimmune Hypophysitis

作　　者：翟笑 朱惠娟 ZHAI Xiao;ZHU Huijuan(Key Laboratory of Endocrinology of National Health Commission,Department of Endocrinology,State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital,Chinese Academy of Medical Science&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,疑难重症及罕见病国家重点实验室,北京100730

出　　处：《罕见病研究》2022年第1期45-50,共6页Journal of Rare Diseases

基　　金：中国医学科学院医学与健康科技创新工程项目(2021⁃I2M⁃1⁃003)。

摘　　要：自身免疫性垂体炎是自身免疫介导侵犯垂体的罕见疾病,其中育龄期女性好发的淋巴细胞性垂体炎最常见。常见临床表现包括头痛、视功能障碍、垂体功能减低等,垂体核磁成像可见弥漫增大、可被增强的鞍内占位。病理是自身免疫性垂体炎诊断的金标准,但因手术创伤和可能的垂体功能损害需谨慎选择。多结合患者典型的临床表现、影像学特征进行临床诊断,糖皮质激素治疗有效是自身免疫性垂体炎临床诊断依据之一。足疗程糖皮质激素治疗淋巴细胞垂体炎有良好的治疗效果,有效改善临床症状、缓解占位效应,促进垂体功能恢复。部分类型的自身免疫性垂体炎需手术治疗。自身免疫性垂体炎的发病机制、诊断标记物和治疗靶点有待深入研究。Autoimmune hypophysitis is a rare disorder characterized by autoimmune⁃mediated in⁃flammation of the pituitary,among which lymphocytic hypophysitis is the most common type that occurs mainly in young women of reproductive ages.Its common clinical manifestation includes headache,vis⁃ual dysfunction,hypopituitarism and etc.Typical magnetic resonance imaging shows diffuse homogenous enlargement of the pituitary gland with gadolinium enhancement and stalk thickening.Pituitary biopsy with histopathological examination is the gold standard for the diagnosis of autoimmune hypophysitis,but it should be performed with extra care in that transsphenoidal surgery is invasive and may cause pitu⁃itary insufficiency.Closely relating to clinical manifestation of the patients and the radiological features is advised for diagnosis.Good response to glucocorticoid therapy also help confirm the diagnosis.Full course of pharmacological glucocorticoid treatment can effectively control the clinical symptoms,reduce the mass effects,and promote the resumption of pituitary functions.Surgery is usually necessary for xan⁃thomatous andgranulomatous hypophysitis.Pathophysiological mechanism,diagnostic biomarkers,and need studying further.

关 键 词：自身免疫性垂体炎 淋巴细胞性垂体炎 

分 类 号：R584[医药卫生—内分泌]