Interpretation of Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update of the International Pituitary Society——Diagnosis

doi:10.12114/j.issn.1007-9572.2022.0190

Chinese General Practice ›› 2022, Vol. 25 ›› Issue (20): 2435-2442.DOI: 10.12114/j.issn.1007-9572.2022.0190

Special Issue: 指南/共识最新文章合辑

• Guide Interpretation • Next Articles

Interpretation of Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update of the International Pituitary Society——Diagnosis

¹Department of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu 610041, China
²Institute of Pituitary Adenomas and Related Diseases Center, West China Hospital, Sichuan University, Chengdu 610041, China

Received:2022-02-15 Revised:2022-03-20 Published:2022-07-15 Online:2022-05-19
Contact: Yerong YU
About author:
TAN H W, TANG Y, YU Y R, et al. Interpretation of Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update of the International Pituitary Society——diagnosis[J]. Chinese General Practice, 2022, 25 (20) : 2435-2442.

国际垂体协会《库欣病的诊断和管理共识（更新版）》解读——诊断篇

¹610041　四川省成都市，四川大学华西医院内分泌代谢科
²610041　四川省成都市，四川大学华西医院垂体瘤及相关疾病诊疗中心

通讯作者: 余叶蓉
作者简介:
谭惠文，唐宇，余叶蓉，等.国际垂体协会《库欣病的诊断和管理共识（更新版）》解读——诊断篇[J].中国全科医学，2022，25（20）：2435-2442. [www.chinagp.net] 作者贡献：谭惠文、余叶蓉提出主要研究内容及目的，负责文章的构思与设计；余叶蓉、李建薇、李佳琦、安振梅、王椿、魏懿进行研究的实施与可行性分析；谭惠文、唐宇进行文献整理、论文撰写和修改，绘制图表；余叶蓉负责文章的质量控制及审校、监督管理。
基金资助:
四川大学华西医院学科卓越发展1·3·5工程临床研究孵化项目(2020HXFH034); 四川省卫生健康委员会项目(20PJ046)

Abstract

Abstract:

Cushing disease, the most common cause of endogenous Cushing's syndrome characterized by hypercortisolemia, is a clinical syndrome caused by adrenocorticotropic hormone (ACTH) ——secreting pituitary adenomas. The excessive secretion of ACTH in Cushing's disease stimulates bilateral adrenal hyperplasia and causes hypercortisolemia, which can lead to a series of severe clinical syndromes such as electrolyte imbalance, glucose and lipid metabolism disorders, involving multiple organs and systems in the body. The clinical symptoms of Cushing's disease are complex and diverse, and its diagnosis and treatment are extremely challenging. In December 2021, the International Pituitary Society released the Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update, which focuses on the complications and comorbidities of Cushing's disease, such as hypercoagulability, cardiovascular disease, metabolic bone disease, growth hormone deficiency and infection, etc.; discusses the application of the latest evidence in clinical practice, with a particular focus on new treatment and screening options, diagnostic algorithms, and best practices for preventing disease recurrence. Based on the latest evidence-based medical evidence, this article focuses on the interpretation of Cushing's disease——related laboratory examinations, imaging examinations, diagnosis and treatment management procedures, and recurrence monitoring discussed in the Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update, in order to improve the general practitioners and specialists'understanding of this syndrome and improve the prognosis of patients with Cushing's disease.

Key words: Cushing syndrome, Cushing disease, Diagnostic and treatment guideline, Pituitary adenoma, Diagnostic techniques and procedures, Therapy

摘要：

库欣病是以高皮质醇血症为特征的内源性库欣综合征最常见的病因，是由分泌促肾上腺皮质激素（ACTH）的垂体腺瘤引起的临床综合征。库欣病过度分泌的ACTH刺激双侧肾上腺皮质增生并引起高皮质醇血症，可导致电解质紊乱，糖、脂代谢紊乱等一系列严重的临床症候群，累及全身多个脏器及系统。库欣病临床症状复杂多样，其诊断和治疗极具挑战性。国际垂体协会在2021年12月发布了《库欣病的诊断和管理共识（更新版）》，该指南更新关注库欣病的并发症和合并症，诸如高凝状态、心血管疾病、骨代谢疾病、生长激素缺乏及感染等；讨论了最新证据在临床实践中的应用，尤其侧重于新的治疗选择、筛查、诊断算法以及防治疾病复发的最佳实践。本文立足于最新的循证医学证据，对《库欣病的诊断和管理共识（更新版）》讨论的库欣病相关的实验室检查、影像学检查、诊断和治疗管理流程及复发监测等多方面进行重点解读，以期提高广大全科及专科医师对库欣病的理解，更好地指导规范化的诊疗，改善库欣病患者预后。

关键词: 库欣综合征, 库欣病, 诊疗指南, 垂体腺瘤, 诊断技术和方法, 治疗

Huiwen TAN,Yu TANG,Yerong YU, et al. Interpretation of Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update of the International Pituitary Society——Diagnosis[J]. Chinese General Practice, 2022, 25(20): 2435-2442. DOI: 10.12114/j.issn.1007-9572.2022.0190.
谭惠文,唐宇,余叶蓉等. 国际垂体协会《库欣病的诊断和管理共识（更新版）》解读——诊断篇[J]. 中国全科医学, 2022, 25(20): 2435-2442. DOI: 10.12114/j.issn.1007-9572.2022.0190.

Figures/Tables 2

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检查项目		灵敏度（%）	特异度（%）	优、缺点
CS诊断指标
	1 mg-DST	98	81	优点：阴性预测值高；易于医疗人员管理缺点：假阳性率高；地塞米松代谢可影响测定结果；其他药物干扰
	24 hUFC	91.0	81.5	优点：参考范围广缺点：操作繁琐；样本之间的多变性可能为50%，需要留取2~3个样本
	LNSC	97.0	97.5	优点：患者易于操作；应提醒患者在收集唾液样本15 min前不要进食、饮水、吸烟或刷牙缺点：多变性；根据参考实验室的不同，临界值会有很大的不同；可能被局部氢化可的松污染；并非所有中心均能检测
CD复发监测
	LNSC	75~90	93~95	优点：多数患者LNSC的异常早于DST和24 hUFC的异常缺点：多变性；易出现正常或阴性结果
	24 hUFC	68	100	优点：相对便捷缺点：大约50%存在变异；容易受体积计量影响而导致异常结果
	DDAVP刺激试验	68	95	优点：可预测存在促肾上腺皮质激素腺瘤；临床腺瘤复发前可以变阳性缺点：多次采血，动态密集检测
	1 mg-DST	NA	NA	优点：可能会在24 hUFC之前出现异常缺点：评估复发效用方面的证据有限

检查项目		灵敏度（%）	特异度（%）	优、缺点
CS诊断指标
	1 mg-DST	98	81	优点：阴性预测值高；易于医疗人员管理缺点：假阳性率高；地塞米松代谢可影响测定结果；其他药物干扰
	24 hUFC	91.0	81.5	优点：参考范围广缺点：操作繁琐；样本之间的多变性可能为50%，需要留取2~3个样本
	LNSC	97.0	97.5	优点：患者易于操作；应提醒患者在收集唾液样本15 min前不要进食、饮水、吸烟或刷牙缺点：多变性；根据参考实验室的不同，临界值会有很大的不同；可能被局部氢化可的松污染；并非所有中心均能检测
CD复发监测
	LNSC	75~90	93~95	优点：多数患者LNSC的异常早于DST和24 hUFC的异常缺点：多变性；易出现正常或阴性结果
	24 hUFC	68	100	优点：相对便捷缺点：大约50%存在变异；容易受体积计量影响而导致异常结果
	DDAVP刺激试验	68	95	优点：可预测存在促肾上腺皮质激素腺瘤；临床腺瘤复发前可以变阳性缺点：多次采血，动态密集检测
	1 mg-DST	NA	NA	优点：可能会在24 hUFC之前出现异常缺点：评估复发效用方面的证据有限

Interpretation of Consensus on Diagnosis and Management of Cushing's Disease: a Guideline Update of the International Pituitary Society——Diagnosis

国际垂体协会《库欣病的诊断和管理共识（更新版）》解读——诊断篇

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