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

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

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。

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.