成人肾上腺皮质功能减退症病因构成及临床特点单中心10年回顾

A 10-year Review of the Etiology and Clinical Features of Adult Adrenal Insufficiency in a Single Center

目的 分析成人肾上腺皮质功能减退症(adrenocortical insufficiency, AI)的病因构成及临床特点。方法 回顾分析我院2011年4月~2021年9月57例AI的临床资料,根据AI定位诊断分为原发性肾上腺皮质功能减退症(primary adrenal insufficiency, PAI)组(n=21)和继发性肾上腺皮质功能减退症(secondary adrenal insufficiency, SAI)组(n=36),对病因进行分析并总结临床特点。结果 共57例AI纳入本研究,最常见的症状为乏力(70.2%,40/57)、消瘦(42.1%,21/57)。PAI组患者盐皮质激素缺乏,低钠血症更常见,皮肤色素沉着是特异性体征。SAI组患者贫血更常见,可伴其他垂体内分泌功能减退的症状。PAI组病因构成:肾上腺结核9例(42.9%,9/21),基因缺陷6例(28.6%,6/21),恶性肿瘤3例(14.3%,3/21),肾上腺出血2例(9.5%,2/21),自身免疫性多内分泌腺综合征Ⅱ型1例(4.8%,1/21)。SAI组病因构成:希恩综合征7例(19.4%,7/36),鞍区手术后7例(19.4%,7/36),垂体瘤压迫8例(22.2%,8/36),空泡蝶鞍7例(19.4%,7/36),自身免疫性垂体炎1例(2.8%,1/36),恶性肿瘤垂体转移1例(2.8%,1/36),免疫检查点抑制剂相关性垂体炎5例(13.9%,5/36)。2016年前,PAI的主要病因为肾上腺结核(63.6%,7/11),SAI为希恩综合征(57.1%,4/7);2017年后,肾上腺结核(20.0%,2/10)和希恩综合征(10.3%,3/29)占比下降,PAI和SAI的病因呈多样化。结论 近10年AI病因构成发生变化,结核和希恩综合征构成比明显下降,手术、药物所致病例明显增加。提高对AI患者临床特点(症状、体征、实验室检查、影像学)和病因的认识,对拟行肾上腺或垂体手术的患者围手术期开展多学科管理。

Objective To analyze the etiology and clinical characteristics of adult patients with adrenocortical insufficiency(AI).Methods A retrospective analysis on clinical and laboratory features of 57 patients with AI from April 2011 to September 2021 in our hospital was performed.The patients were divided into primary adrenal insufficiency(PAI)group and secondary adrenal insufficiency(SAI)group according to its localization diagnosis.The etiology was analyzed and the clinical characteristics were summarized.Results A total of 57 patients with AI were included in this study.The most common symptoms were fatigue(70.2%,40/57)and wasting(42.1%,21/57).In the PAI group,mineralocorticoid deficiency and hyponatremia were more common,and skin hyperpigmentation was a specific sign.Anemia was more common in patients with SAI,companied with other symptoms of pituitary deficiency such as secondary hypothyroidism.Etiological composition of the PAI group:9 cases of adrenal tuberculosis(42.9%,9/21),6 cases of gene deficiency(28.6%,6/21),3 cases of malignant tumors(14.3%,3/21),2 cases of adrenal hemorrhage(9.5%,2/21),and 1 case of autoimmune polyendocrine syndrome-Ⅱ(4.8%,1/21).Etiological composition of the SAI group:7 cases of Sheehan’s syndrome (19. 4% , 7 /36), 7 cases of post-operation of sellar region (19. 4% , 7 /36), 8 cases of pituitary adenoma compression (22. 2% , 8 /36), 7 cases of empty sella syndrome (19. 4% ,7 /36), 1 case of autoimmune hypophysitis (2. 8% ,1 /36), 1 case of pituitary metastasis of malignancy (2. 8% , 1 /36), and 5 cases of immune checkpoint inhibitors (ICPIs) related hypophysitis (13. 9% , 5 /36). In patients enrolled before 2016, the main causes of PAI and SAI were adrenal tuberculosis (63. 6% ,7 /11) and Sheehan’s syndrome (57. 1% , 4 /7), respectively. After 2017, the incidence of adrenal tuberculosis (20. 0% , 2 /10) and Sheehan’s syndrome (10. 3% , 3 /29) decreased, and the causes of PAI and SAI were diverse. Conclusions In the past 10 years, the etiological composition of AI has changed. The proportion of tuberculosis and Sheehan’s syndrome has decreased significantly, while the cases caused by surgery and drugs have increased significantly. It is important to improve better understanding of the clinical characteristics and etiology of AI and promote perioperative multidisciplinary management of patients undergoing adrenal or pituitary surgery.