Amodru Vincent, Ferriere Amandine, Tabarin Antoine, Castinetti Frederic, Tsagarakis Stylianos, Toth Miklos, Feelders Richard A, Webb Susan M, Reincke Martin, Netea-Maier Romana, Kastelan Darko, Elenkova Atanaska, Maiter Dominique, Ragnarsson Oskar, Santos Alicia, Valassi Elena
IIB-Sant Pau and Department of Endocrinology, Hospital Sant Pau, Dept Medicine, UAB, and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona 08025, Spain.
Aix-Marseille Université, Institut National de la Santé et de la Recherche Médicale INSERM U1251, Marseille Medical Genetics, Marseille, France.
Eur J Endocrinol. 2023 Apr 5;188(4):395-406. doi: 10.1093/ejendo/lvad008.
To evaluate whether age-related differences exist in clinical characteristics, diagnostic approach, and management strategies in patients with Cushing's syndrome (CS) included in the European Registry on Cushing's Syndrome (ERCUSYN).
Cohort study.
We analyzed 1791 patients with CS, of whom 1234 (69%) had pituitary-dependent CS (PIT-CS), 450 (25%) adrenal-dependent CS (ADR-CS), and 107 (6%) had an ectopic source (ECT-CS). According to the WHO criteria, 1616 patients (90.2%) were classified as younger (<65 years old) and 175 (9.8%) as older (≥65 years old).
Older patients were more frequently males and had a lower Body Mass Index (BMI) and waist circumference when compared with the younger. Older patients also had a lower prevalence of skin alterations, depression, hair loss, hirsutism, and reduced libido, but a higher prevalence of muscle weakness, diabetes, hypertension, cardiovascular disease, venous thromboembolism, and bone fractures than younger patients, regardless of sex (P < .01 for all comparisons). Measurement of urinary free cortisol supported the diagnosis of CS less frequently in older patients when compared with the younger (P < .05). An extrasellar macroadenoma (macrocorticotropinoma with extrasellar extension) was more common in older PIT-CS patients than in the younger (P < .01). Older PIT-CS patients more frequently received cortisol-lowering medications and radiotherapy as a first-line treatment, whereas surgery was the preferred approach in the younger (P < .01 for all comparisons). When transsphenoidal surgery was performed, the remission rate was lower in the elderly when compared with their younger counterpart (P < .05).
Older CS patients lack several typical symptoms of hypercortisolism, present with more comorbidities regardless of sex, and are more often conservatively treated.
评估欧洲库欣综合征注册研究(ERCUSYN)中库欣综合征(CS)患者在临床特征、诊断方法和管理策略方面是否存在年龄相关差异。
队列研究。
我们分析了1791例CS患者,其中1234例(69%)为垂体依赖性CS(PIT-CS),450例(25%)为肾上腺依赖性CS(ADR-CS),107例(6%)有异位来源(ECT-CS)。根据世界卫生组织标准,1616例患者(90.2%)被分类为较年轻(<65岁),175例(9.8%)为较年长(≥65岁)。
与较年轻患者相比,较年长患者男性更常见,体重指数(BMI)和腰围更低。较年长患者皮肤改变、抑郁、脱发、多毛症和性欲减退的患病率也较低,但无论性别,肌肉无力、糖尿病、高血压、心血管疾病、静脉血栓栓塞和骨折的患病率均高于较年轻患者(所有比较P<0.01)。与较年轻患者相比,老年患者尿游离皮质醇测定支持CS诊断的频率较低(P<0.05)。鞍外大腺瘤(伴有鞍外扩展的大促肾上腺皮质激素瘤)在老年PIT-CS患者中比在年轻患者中更常见(P<0.01)。老年PIT-CS患者更常接受降低皮质醇的药物和放疗作为一线治疗,而手术是年轻患者的首选方法(所有比较P<0.01)。当进行经蝶窦手术时,老年人的缓解率低于年轻患者(P<0.05)。
老年CS患者缺乏几种典型的皮质醇增多症症状,无论性别均伴有更多合并症,且更常接受保守治疗。