探讨外周DDAVP刺激试验联合HDDST在库欣病与异位ACTH综合征鉴别诊断中的价值

杨彩彩; 郭清华; 窦京涛; 杜锦; 王先令; 吕朝晖; 谷伟军; 程愈; 巴建明; 母义明

doi:10.3969/j.issn.2095-5227.2021.10.001

探讨外周DDAVP刺激试验联合HDDST在库欣病与异位ACTH综合征鉴别诊断中的价值

Value of peripheral DDAVP stimulation test combined with HDDST in differential diagnosis of Cushing's disease and ectopic ACTH syndrome

摘要

摘要:
背景促肾上腺皮质激素(adrenocorticotropic hormone，ACTH)依赖性库欣综合征的常见病因为库欣病(Cushing's disease，CD)和异位ACTH综合征(ectopic ACTH syndrome，EAS)，岩下窦静脉分段采血(bilateral inferior petrosal sinus sampling，BIPSS)是鉴别两者的金标准，但因其有创性和技术要求高难以临床广泛开展，而大剂量地塞米松抑制试验(high-dose dexamethasone suppression test，HDDST)及外周血1-去氨基-8-D-精氨酸血管加压素(desmopressin，DDAVP)刺激试验操作较为简单，可广泛开展。
目的探讨外周血DDAVP刺激试验联合HDDST鉴别诊断库欣病和异位ACTH综合征的价值。
方法回顾性分析2016年1月- 2019年12月在解放军总医院第一医学中心内分泌科经术后病理、临床指标、BIPSS确诊的ACTH依赖性库欣综合征病例的临床资料，分析患者血ACTH-皮质醇节律、24 h尿游离皮质醇(24 h urinary free cortisol，24 h UFC)、外周DDAVP刺激试验后血ACTH及血皮质醇变化和HDDST后血、尿皮质醇变化，探讨外周DDAVP刺激试验和(或)经典HDDST不同切点鉴别诊断CD与EAS的敏感度和特异性。
结果 119例ACTH依赖性库欣综合征患者中，CD 105例，男性10例，女性95例，平均年龄(38.20±12.52)岁；EAS 14例，男性8例，女性6例，平均年龄(44.86±17.17)岁。以外周DDAVP刺激试验后血ACTH升高幅度≥35%为切点，鉴别诊断CD与EAS的敏感度和特异性分别为97.9%和55.6%；以经典HDDST血和尿皮质醇抑制率均度≥50%为切点，鉴别CD与EAS的敏感度和特异性分别为62.0%和90.0%；联合外周DDAVP刺激试验血ACTH升高≥35%和HDDST试验血皮质醇(或尿游离皮质醇)抑制率≥50%为切点，鉴别ACTH依赖性库欣综合征的敏感度和特异性分别为73.3%和87.5%。
结论以外周DDAVP刺激试验作为ACTH依赖性库欣综合征的定位试验，敏感度较高，但特异性较低；以HDDST作为ACTH依赖性库欣综合征的定位试验，敏感度较低，但特异性较高；两个试验联合诊断，可提高鉴别库欣病和异位ACTH综合征的诊断效能。

Abstract:
Background The common causes of adrenocorticotropic hormone (ACTH) dependent Cushing syndrome include Cushing’s disease (CD) and ectopic ACTH syndrome (EAS). The gold standard for distinguishing these two diseases is the bilateral inferior petrosal sinus sampling (BIPSS), however, it can not be widely carried out due to its invasiveness and technical demand. The high-dose dexamethasone suppression test (HDDST) and peripheral blood 1-deamino-8-D-arginine vasopressin (DDAVP) stimulation test are relatively simple and can be safely performed.
Objective To investigate the clinical value of peripheral DDAVP stimulation test combined with high-dose dexamethasone suppression test in differential diagnosis of EAS and CD.
Methods A retrospective analysis was conducted for the clinical data about patients with ACTH-dependent Cushing’s syndrome diagnosed based on pathology, clinical indicators, and BIPSS in the department of endocrinology, the First Medical Center of Chinese PLA General Hospital from January 2016 to December 2019. The ACTH-cortisol level, 24 h urinary free cortisol (24 h UFC), changes in blood ACTH and blood cortisol after peripheral DDAVP stimulation test and HDDST were analyzed. The sensitivity and specificity of the peripheral DDAVP stimulation test and/or classic HDDST in the differential diagnosis between CD and EAS were explored.
Results Among the 119 patients with ACTH-dependent Cushing’s syndrome, 105 cases had Cushing’s disease (10 male , 95 female, with an average age of 38.20±12.52 years); 14 cases had ectopic ACTH syndrome (8 male, 6 female, with an average age of 44.86±17.17 years). When the blood ACTH increase ≥35% in the peripheral DDAVP stimulation test was used as the cut-off point, the sensitivity and specificity of the differential diagnosis of CD and EAS was 97.9% and 55.6%, respectively; if the blood and urine cortisol inhibition rate of ≥ 50% in classic HDDST was used as the cut point, the sensitivity and specificity was 62.0% and 90.0%, respectively; if combined ACTH increase ≥ 35% in peripheral DDAVP stimulation test with blood cortisol inhibition rate (or urine Free cortisol inhibition rate) ≥50% in HDDST, the sensitivity and specificity was 73.3% and 87.5%, respectively.
Conclusion Peripheral DDAVP stimulation test shows high sensitivity but low specificity for distinguishing EAS and CD; wheares HDDST shows low sensitivity, and high specificity; if the two tests are combined, the diagnostic performance improved.

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