Alexandraki Krystallenia I, Kaltsas Gregory A, Isidori Andrea M, Akker Scott A, Drake William M, Chew Shern L, Monson John P, Besser G Michael, Grossman Ashley B
Department of Endocrinology, St Bartholomew's Hospital, London EC1A 7BE, UK.
Eur J Endocrinol. 2009 Jun;160(6):1011-8. doi: 10.1530/EJE-09-0046. Epub 2009 Mar 16.
Cyclical Cushing's syndrome may render the diagnosis and management of Cushing's disease difficult. The aim of the present study was to investigate the prevalence of cyclicity and variability in patients with Cushing's disease, and to identify putative distinctive features.
Retrospective case-note study.
We analysed the case records of 201 patients with Cushing's disease in a retrospective case-note study. Cyclicity was considered as the presence of at least one cycle, defined as a clinical and/or biochemical hypercortisolaemic peak followed by clinical and biochemical remission, followed by a new clinical and/or biochemical hypercortisolaemic peak. The fluctuations of mean serum cortisol levels, as assessed by a 5-point cortisol day curve, defined the variability.
Thirty (14.9%; 26 females) patients had evidence of cyclicity/variability. 'Cycling' patients were older but no difference in sex or paediatric distribution was revealed between 'cycling' and 'non-cycling' patients. The median number of cycles was two for each patient, and 4 years was the median intercyclic period. A trend to lower cure rate post-neurosurgery and lower adenoma identification was observed in 'cycling' compared with 'non-cycling' patients. In multivariate analysis, older patients, longer follow-up, female sex and no histological identification of the adenoma were associated with an increased risk of cyclic disease.
This large population study reveals that cyclicity/variability is not an infrequent phenomenon in patients with Cushing's disease, with a minimum prevalence of 15%. Physicians should be alert since it can lead to frequent problems in diagnosis and management, and no specific features can be used as markers.
周期性库欣综合征可能会使库欣病的诊断和治疗变得困难。本研究的目的是调查库欣病患者中周期性和变异性的发生率，并确定可能的独特特征。
回顾性病例记录研究。
我们在一项回顾性病例记录研究中分析了201例库欣病患者的病例记录。周期性被定义为至少存在一个周期，即临床和/或生化高皮质醇血症高峰后临床和生化缓解，随后出现新的临床和/或生化高皮质醇血症高峰。通过5点皮质醇日曲线评估的平均血清皮质醇水平波动定义为变异性。
30例（14.9%；26例女性）患者有周期性/变异性证据。“周期性”患者年龄较大，但“周期性”和“非周期性”患者在性别或儿科分布上没有差异。每位患者的周期中位数为2个，周期间期中位数为4年。与“非周期性”患者相比，“周期性”患者神经外科手术后治愈率较低且腺瘤识别率较低。在多变量分析中，年龄较大、随访时间较长、女性以及腺瘤无组织学识别与周期性疾病风险增加相关。
这项大型人群研究表明，周期性/变异性在库欣病患者中并非罕见现象，最低发生率为15%。医生应保持警惕，因为它可能导致诊断和治疗中频繁出现问题，且没有特定特征可作为标志物。
