Mifepristone in the treatment of the ectopic adrenocorticotropic hormone syndrome.
Mifepristone in the treatment of the ectopic adrenocorticotropic hormone syndrome.
复制标题
米非司酮治疗异位促肾上腺皮质激素综合征。
DOI：
10.1111/cen.13818
复制
发表时间：
2018
影响因子：
3.2
通讯作者：
Auchus,RichardJ
中科院分区：
文献类型：
--
作者：
Wannachalee,Taweesak;Turcu,AdinaF;Auchus,RichardJ
目的米非司酮是一种糖皮质激素受体拮抗剂，在手术治疗不可行的情况下，可用于治疗异位促肾上腺皮质激素综合征（EAS）患者的高皮质醇症。用米非司酮治疗的EAS患者的结果仅限于孤立病例的报道。我们的目的是确定米非司酮治疗EAS的疗效和局限性，并与接受双侧肾上腺切除术的患者的结果进行比较。方法对1997 - 2017年密歇根大学EAS患者进行回顾性队列研究。结果55例EAS患者中，米非司酮治疗16例，其中8例为神经内分泌肿瘤，2例为癌，6例为隐匿性肿瘤。米非司酮治疗最常由精神病、血糖不控制和/或高血压引起。中位维持剂量为600mg /d。3/3的患者在48小时内精神症状得到改善，14/16的患者血糖控制得到改善。中位治疗时间为9个月，3例患者治疗时间超过24个月。在接受米非司酮治疗的EAS患者与双侧肾上腺切除术患者之间，24个月的总生存率相当（N = 12） （P=0.6）。结论司米司酮治疗EAS 2年以上有效，生存率与双侧肾上腺切除术患者无明显差异。对低钾血症和高血压进行积极的联合治疗是必要的。
ObjectivesMifepristone, a glucocorticoid receptor antagonist, can be used to manage hypercortisolism in patients with ectopic adrenocorticotropic hormone syndrome (EAS) when surgical cure is not feasible. Outcomes of EAS patients treated with mifepristone have been limited to reports of isolated cases. We aimed to determine the efficacy and limitations of mifepristone in the treatment of EAS and to compare outcomes with those of patients who underwent bilateral adrenalectomy.MethodA retrospective cohort study of EAS patients from the University of Michigan between 1997 and 2017 was conducted.ResultsOf the 55 patients with EAS, 16 were treated with mifepristone: eight neuroendocrine tumours, two carcinomas and six occult tumours. Treatment with mifepristone was most commonly prompted by psychosis, uncontrolled glucose and/or hypertension. The median maintenance dose was 600 mg/d. Amelioration of psychosis was observed within 48 hours in 3/3 patients, and the glycaemic control was improved in 14/16 patients. The median duration of treatment was 9 months, and three patients were treated for more than 24 months. The overall survival at 24 months was equivalent between patients with EAS treated with mifepristone vs bilateral adrenalectomy (N = 12) (P=0.6).ConclusionsMifepristone is effective in treating EAS for over 2 years, and survival was not different from that of patients treated with bilateral adrenalectomy. Aggressive concomitant therapy for hypokalaemia and hypertension is necessary.