Ragucci Enzo, Nguyen Dat, Lamerson Michele, Moraitis Andreas G
Diabetes and Endocrinology Consultants, 2 Crosfield Ave, No. 204, West Nyack, NY 10994, USA.
Corcept Therapeutics, 149 Commonwealth Drive, Menlo Park, CA 94025, USA.
Case Rep Endocrinol. 2017;2017:6161348. doi: 10.1155/2017/6161348. Epub 2017 Nov 19.
Cushing syndrome (CS), a complex, multisystemic condition resulting from prolonged exposure to cortisol, is frequently associated with nonalcoholic fatty liver disease (NAFLD). In patients with adrenal adenoma(s) and NAFLD, it is essential to rule out coexisting endocrine disorders like CS, so that the underlying condition can be properly addressed. We report a case of a 49-year-old woman with a history of hypertension, prediabetes, dyslipidemia, biopsy-confirmed steatohepatitis, and benign adrenal adenoma, who was referred for endocrine work-up for persistent weight gain. Overt Cushing features were absent. Biochemical evaluation revealed nonsuppressed cortisol on multiple 1-mg dexamethasone suppression tests, suppressed adrenocorticotropic hormone, and low dehydroepiandrosterone sulfate. The patient initially declined surgery and was treated with mifepristone, a competitive glucocorticoid receptor antagonist. In addition to improvements in weight and hypertension, substantial reductions in her liver enzymes were noted, with complete normalization by 20 weeks of therapy. This case suggests that autonomous cortisol secretion from adrenal adenoma(s) could contribute to the metabolic and liver abnormalities in patients with NAFLD. In conclusion, successful management of CS with mifepristone led to marked improvement in the liver enzymes of a patient with long-standing NAFLD.
库欣综合征(CS)是一种因长期暴露于皮质醇而导致的复杂多系统疾病,常与非酒精性脂肪性肝病(NAFLD)相关。对于患有肾上腺腺瘤和NAFLD的患者,排除如CS等并存的内分泌疾病至关重要,以便能恰当处理潜在病情。我们报告一例49岁女性病例,她有高血压、糖尿病前期、血脂异常、活检证实的脂肪性肝炎病史以及良性肾上腺腺瘤,因持续体重增加而被转诊进行内分泌检查。未发现明显的库欣特征。生化评估显示在多次1毫克地塞米松抑制试验中皮质醇未被抑制、促肾上腺皮质激素被抑制以及硫酸脱氢表雄酮水平低。该患者最初拒绝手术,接受了米非司酮治疗,米非司酮是一种竞争性糖皮质激素受体拮抗剂。除体重和高血压有所改善外,还注意到她的肝酶大幅降低,治疗20周时完全恢复正常。该病例表明肾上腺腺瘤自主分泌皮质醇可能导致NAFLD患者出现代谢和肝脏异常。总之,用米非司酮成功治疗CS使一名长期患有NAFLD患者的肝酶显著改善。