Cuevas-Ramos Daniel, Fleseriu Maria
Department of MedicinePituitary Center, Cedars-Sinai Medical Center, Los Angeles, California, USANeuroendocrinology ClinicDepartment of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, MexicoDepartments of Medicine and Neurological Surgeryand Northwest Pituitary Center, Oregon Health & Science University, 3181 SW Sam Jackson Park Road (BTE 472), Portland, Oregon 97239, USA Department of MedicinePituitary Center, Cedars-Sinai Medical Center, Los Angeles, California, USANeuroendocrinology ClinicDepartment of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, MexicoDepartments of Medicine and Neurological Surgeryand Northwest Pituitary Center, Oregon Health & Science University, 3181 SW Sam Jackson Park Road (BTE 472), Portland, Oregon 97239, USA.
Department of MedicinePituitary Center, Cedars-Sinai Medical Center, Los Angeles, California, USANeuroendocrinology ClinicDepartment of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, MexicoDepartments of Medicine and Neurological Surgeryand Northwest Pituitary Center, Oregon Health & Science University, 3181 SW Sam Jackson Park Road (BTE 472), Portland, Oregon 97239, USA
J Endocrinol. 2014 Nov;223(2):R19-39. doi: 10.1530/JOE-14-0300. Epub 2014 Aug 18.
Cushing's disease (CD) is characterized by an ACTH-producing anterior corticotrope pituitary adenoma. If hypothalamus-pituitary-adrenal (HPA) axis physiology is disrupted, ACTH secretion increases, which in turn stimulates adrenocortical steroidogenesis and cortisol production. Medical treatment plays an important role for patients with persistent disease after surgery, for those in whom surgery is not feasible, or while awaiting effects of radiation. Multiple drugs, with different mechanisms of action and variable efficacy and tolerability for controlling the deleterious effects of chronic glucocorticoid excess, are available. The molecular basis and clinical data for centrally acting drugs, adrenal steroidogenesis inhibitors, and glucocorticoid receptor antagonists are reviewed, as are potential novel molecules and future possible targets for CD treatment. Although progress has been made in the understanding of specific corticotrope adenoma receptor physiology and recent clinical studies have detected improved effects with a combined medical therapy approach, there is a clear need for a more efficacious and better-tolerated medical therapy for patients with CD. A better understanding of the molecular mechanisms in CD and of HPA axis physiology should advance the development of new drugs in the future.
库欣病(CD)的特征是产生促肾上腺皮质激素(ACTH)的垂体前叶促肾上腺皮质激素细胞腺瘤。如果下丘脑-垂体-肾上腺(HPA)轴生理功能受到干扰,ACTH分泌增加,进而刺激肾上腺皮质类固醇生成和皮质醇产生。对于术后疾病持续存在的患者、手术不可行的患者或等待放疗效果的患者,药物治疗起着重要作用。有多种药物可用于控制慢性糖皮质激素过量的有害影响,它们的作用机制不同,疗效和耐受性也各不相同。本文综述了中枢作用药物、肾上腺类固醇生成抑制剂和糖皮质激素受体拮抗剂的分子基础及临床数据,以及CD治疗的潜在新分子和未来可能的靶点。尽管在理解特定促肾上腺皮质激素细胞腺瘤受体生理学方面取得了进展,并且最近的临床研究发现联合药物治疗方法有更好的效果,但显然需要为CD患者开发一种更有效且耐受性更好的药物治疗。更好地理解CD中的分子机制和HPA轴生理学应能推动未来新药的开发。