Cuevas-Ramos Daniel, Fleseriu Maria
Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Salvador Zubiran National Institute of Medical Sciences and Nutrition, Tlalpan, Mexico City, Mexico -
Minerva Endocrinol. 2016 Sep;41(3):324-40. Epub 2016 Mar 15.
Cushing's Syndrome (CS) is a serious endocrine disease that results from the adverse clinical consequences of chronic exposure to high levels of glucocorticoids. Most patients with endogenous CS have an adrenocorticotropin (ACTH)-secreting pituitary corticotroph adenoma, i.e. Cushing's Disease (CD). The first-line therapy for CD is transsphenoidal pituitary surgery. If tumor removal is incomplete or unsuccessful, persistent hypercortisolism will require further treatment. Repeat surgery, medical therapy, radiation and bilateral adrenalectomy are all second line therapy options; however, medical therapy can be also used as first line therapy in patients who cannot undergo surgery, or to decrease cortisol values and/or improve co-morbidities. Medications used in the treatment of CD, classified into three groups: pituitary directed drugs, adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers, are reviewed. Future 'on the horizon' treatment options are also discussed.
库欣综合征(CS)是一种严重的内分泌疾病,由长期暴露于高水平糖皮质激素的不良临床后果引起。大多数内源性CS患者患有分泌促肾上腺皮质激素(ACTH)的垂体促肾上腺皮质激素腺瘤,即库欣病(CD)。CD的一线治疗方法是经蝶窦垂体手术。如果肿瘤切除不完全或不成功,持续性高皮质醇血症将需要进一步治疗。再次手术、药物治疗、放疗和双侧肾上腺切除术都是二线治疗选择;然而,药物治疗也可用于不能接受手术的患者,或降低皮质醇值和/或改善合并症。本文综述了用于治疗CD的药物,这些药物分为三类:垂体靶向药物、肾上腺类固醇生成抑制剂和糖皮质激素受体阻滞剂。还讨论了未来“即将出现”的治疗选择。