Department of Endocrinology and Reference Center for Rare Pituitary Diseases La Timone Hospital, Aix-Marseille University, Marseille, France.
Orphanet J Rare Dis. 2012 Jun 18;7:41. doi: 10.1186/1750-1172-7-41.
Cushing's disease, or pituitary ACTH dependent Cushing's syndrome, is a rare disease responsible for increased morbidity and mortality. Signs and symptoms of hypercortisolism are usually non specific: obesity, signs of protein wasting, increased blood pressure, variable levels of hirsutism. Diagnosis is frequently difficult, and requires a strict algorithm. First-line treatment is based on transsphenoidal surgery, which cures 80% of ACTH-secreting microadenomas. The rate of remission is lower in macroadenomas. Other therapeutic modalities including anticortisolic drugs, radiation techniques or bilateral adrenalectomy will thus be necessary to avoid long-term risks (metabolic syndrome, osteoporosis, cardiovascular disease) of hypercortisolism. This review summarizes potential pathophysiological mechanisms, diagnostic approaches, and therapies.
库欣病，或垂体 ACTH 依赖性库欣综合征，是一种罕见的疾病，可导致发病率和死亡率增加。皮质醇增多症的体征和症状通常是非特异性的：肥胖、蛋白质消耗迹象、血压升高、多毛症程度不一。诊断通常较为困难，需要严格的算法。一线治疗基于经蝶窦手术，该方法可治愈 80%的 ACTH 分泌微腺瘤。大腺瘤的缓解率较低。因此，需要其他治疗方法，包括皮质类固醇抑制剂、放射技术或双侧肾上腺切除术，以避免皮质醇增多症的长期风险（代谢综合征、骨质疏松症、心血管疾病）。本文综述了潜在的病理生理学机制、诊断方法和治疗方法。
