Papoian Vardan, Biller Beverly M K, Webb Susan M, Campbell Karen K, Hodin Richard A, Phitayakorn Roy
Endocr Pract. 2016 Jan;22(1):51-67. doi: 10.4158/EP15855.OR. Epub 2015 Oct 5.
Excess cortisol production (Cushing syndrome, CS) is a chronic disease affecting many organ systems and impacting quality of life (QoL). This study analyzed factors associated with self-reported QoL, including aspects related to the diagnosis and treatment modalities of CS.
In collaboration with the Cushing's Support and Research Foundation (CSRF), surveys using a validated QoL instrument were sent to CSRF members. Data were analyzed for associations between QoL and demographic, treatment, and disease factors.
A total of 269 patients completed the survey. Respondents were 89.9% female, and the mean age was 48 years (SD 12, range 16-76). Respondents visited a median of 4 physicians (range 1-40) prior to the diagnosis of CS, with a median of 5 years (mean 7, SD 5, range 1-30) to obtain a diagnosis, showing a statistically significant negative correlation (P<.001). In one-quarter of cases, someone other than a physician suggested the diagnosis. Multiple regression analysis demonstrated that remission status, time to diagnosis, radiation therapy, and hypopituitarism were significant predictors of QoL. There was no association between QoL and patient's sex, age, replacement steroid use, having follow-up with an endocrinologist, or surgical approach.
This is one of the largest QoL studies of CS patients and provides information for treatment and education goals. It is notable that early diagnosis and treatment was the major predictor of better QoL after achieving remission from disease, highlighting the need for awareness about the disorder. Patients in remission had better QoL, emphasizing the importance of disease control.
皮质醇分泌过多（库欣综合征，CS）是一种影响多个器官系统并影响生活质量（QoL）的慢性疾病。本研究分析了与自我报告的生活质量相关的因素，包括与CS诊断和治疗方式相关的方面。
与库欣支持与研究基金会（CSRF）合作，使用经过验证的生活质量工具对CSRF成员进行调查。分析了生活质量与人口统计学、治疗和疾病因素之间的关联。
共有269名患者完成了调查。受访者中89.9%为女性，平均年龄为48岁（标准差12，范围16 - 76岁）。受访者在CS诊断前平均看了4位医生（范围1 - 40位），诊断时间中位数为5年（平均7年，标准差5年，范围1 - 30年），两者呈统计学显著负相关（P <.001）。在四分之一的病例中，是医生以外的人提出了诊断。多元回归分析表明，缓解状态、诊断时间、放射治疗和垂体功能减退是生活质量的显著预测因素。生活质量与患者的性别、年龄、使用替代类固醇、是否接受内分泌科医生随访或手术方式之间无关联。
这是关于CS患者最大规模的生活质量研究之一，为治疗和教育目标提供了信息。值得注意的是，早期诊断和治疗是疾病缓解后生活质量改善的主要预测因素，凸显了提高对该疾病认识的必要性。缓解期的患者生活质量更好，强调了疾病控制的重要性。
