Pivonello Rosario, De Martino Maria Cristina, De Leo Monica, Simeoli Chiara, Colao Annamaria
Dipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Università Federico II di Napoli, Via S. Pansini, 5, 80131, Naples, Italy.
Endocrine. 2017 Apr;56(1):10-18. doi: 10.1007/s12020-016-0984-8. Epub 2016 May 17.
Cushing's syndrome is caused by prolonged exposure to elevated cortisol levels. The most common form of endogenous Cushing's syndrome is Cushing's disease, which results from an adrenocorticotropic hormone-secreting pituitary tumour. Cushing's disease is associated with increased mortality, mostly attributable to cardiovascular complications, and a host of comorbidities such as metabolic and skeletal disorders, infections and neuropsychiatric disturbances. As a consequence, Cushing's disease substantially impairs health-related quality of life. It is crucial that the condition is diagnosed as early as possible, and that rapid and effective treatment is initiated in order to limit long-term morbidity and mortality. The initial treatment of choice for Cushing's disease is selective transsphenoidal pituitary surgery; however, the risk of recurrence after initial surgery is high and remains so for many decades after surgery. A particular concern is the growing body of evidence indicating that the negative physical and psychosocial sequelae of chronic hypercortisolism may persist in patients with Cushing's disease even after long-term surgical 'cure'. Current treatment options for post-surgical patients with persistent or recurrent Cushing's disease include second surgery, radiotherapy, bilateral adrenalectomy and medical therapy; however, each approach has its limitations and there is an unmet need for more efficacious treatments. The current review provides an overview of the burden of illness of Cushing's disease, underscoring the need for prompt diagnosis and effective treatment, as well as highlighting the need for better therapies.
库欣综合征是由长期暴露于升高的皮质醇水平引起的。内源性库欣综合征最常见的形式是库欣病，它由分泌促肾上腺皮质激素的垂体肿瘤所致。库欣病与死亡率增加相关，主要归因于心血管并发症以及一系列合并症，如代谢和骨骼疾病、感染及神经精神障碍。因此，库欣病严重损害与健康相关的生活质量。尽早诊断该病并启动快速有效的治疗以限制长期发病率和死亡率至关重要。库欣病的初始治疗选择是选择性经蝶窦垂体手术；然而，初次手术后复发风险很高，且术后数十年仍会如此。一个特别令人担忧的问题是，越来越多的证据表明，即使经过长期手术“治愈”，库欣病患者慢性高皮质醇血症的负面身体和心理社会后遗症可能仍然存在。对于术后持续性或复发性库欣病患者，目前的治疗选择包括二次手术、放疗、双侧肾上腺切除术和药物治疗；然而，每种方法都有其局限性，对更有效治疗方法的需求尚未得到满足。本综述概述了库欣病的疾病负担，强调了及时诊断和有效治疗的必要性，同时突出了对更好治疗方法的需求。
