Institute of Metabolism and System Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom; Centre of Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom; Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.
Department of Ear, Nose and Throat, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.
Best Pract Res Clin Endocrinol Metab. 2021 Jan;35(1):101521. doi: 10.1016/j.beem.2021.101521. Epub 2021 Mar 15.
Endogenous Cushing's syndrome (CS) is a rare endocrine disorder characterised by excess cortisol secretion due to either ACTH-dependent conditions [commonly an ACTH-producing pituitary adenoma (Cushing's disease)] or ACTH-independent causes (with most common aetiology being a benign adrenal adenoma). Overall, the annual incidence of CS ranges between 1.8 and 3.2 cases per million population. Mortality in active CS is elevated compared to the general population, and a number of studies support the view that survival is also compromised even after apparent successful treatment. The main cause of death is cardiovascular disease highlighting the negative impact of cortisol excess on cardiovascular risk factors. Early diagnosis and prompt treatment of the cortisol excess, as well as vigilant monitoring and stringent control of cardiovascular risk factors are key elements for the long-term prognosis of these patients.
内源性库欣综合征（CS）是一种罕见的内分泌疾病，其特征是由于 ACTH 依赖性疾病（常见的是 ACTH 分泌性垂体腺瘤（库欣病））或 ACTH 非依赖性原因（最常见的病因是良性肾上腺腺瘤）导致皮质醇分泌过多。总体而言，CS 的年发病率在每百万人口 1.8 至 3.2 例之间。与普通人群相比，活动期 CS 的死亡率升高，多项研究支持这样一种观点，即即使在明显成功治疗后，生存也会受到影响。死亡的主要原因是心血管疾病，这突显了皮质醇过多对心血管危险因素的负面影响。早期诊断和及时治疗皮质醇过多，以及对心血管危险因素的警惕监测和严格控制是这些患者长期预后的关键因素。
