Ritzel Katrin, Fazel Julia, August Leah, Fedtke Vanessa, Nowak Elisabeth, Vogel Frederick, Braun Leah, Zopp Stephanie, Then Cornelia, Künzel Heike, Reisch Nicole, Zimmermann Petra, Thorsteinsdottir Jun, Schopohl Jochen, Bidlingmaier Martin, Beuschlein Felix, Reincke Martin, Rubinstein German
Department of Medicine IV, LMU University Hospital, LMU Munich, 80336 Munich, Germany.
Department of General, Visceral and Transplantation Surgery, LMU University Hospital, LMU Munich, 81377 Munich, Germany.
J Clin Endocrinol Metab. 2025 Mar 17;110(4):e1038-e1045. doi: 10.1210/clinem/dgae337.
Normalization of hypercortisolism is essential to reduce morbidity and mortality in patients with Cushing's syndrome (CS). The aim of this analysis was to assess biochemical control rates in patients with Cushing's disease (CD), ectopic Cushing's syndrome (ECS), and adrenal Cushing's syndrome (ACS).
Patients with confirmed CS (n = 296) treated in a single tertiary care center were retrospectively analyzed (185 CD, 27 ECS, 84 uni- and bilateral ACS).
First-line treatment led to biochemical control in 82% of the patients. Time to biochemical control (median, interquartile range) was longer in CD (11.0 weeks, 5.6-29.8; P < .05) than in ACS (7.7 weeks, 4.1-17.1) and ECS (5.6 weeks, 4.1-23.3). Disease persistence or recurrence after first-line therapy was observed more often in CD (24% and 18%; P < .05) than in ECS (15% and 15%) and ACS (6% and 4%). Total time in hypercortisolism since diagnosis was significantly shorter in patients with CD diagnosed since 2013, after specialized patient care was implemented, compared to patients diagnosed before 2013 (13.5 weeks vs 26.1 weeks; P < .0070). Control of hypercortisolism at last follow-up (76 months, 38-163) was achieved in 94% of patients with ACS, 100% of patients with ECS, and 92% of patients with CD.
Biochemical control can be achieved in most patients with different subtypes of CS within a reasonable time frame. Control of hypercortisolism has improved over time.
高皮质醇血症的正常化对于降低库欣综合征（CS）患者的发病率和死亡率至关重要。本分析的目的是评估库欣病（CD）、异位库欣综合征（ECS）和肾上腺库欣综合征（ACS）患者的生化控制率。
对在单一三级医疗中心接受治疗的确诊CS患者（n = 296）进行回顾性分析（185例CD、27例ECS、84例单侧和双侧ACS）。
一线治疗使82%的患者实现生化控制。CD患者达到生化控制的时间（中位数，四分位间距）（11.0周，5.6 - 29.8；P <.05）比ACS患者（7.7周，4.1 - 17.1）和ECS患者（5.6周，4.1 - 23.3）更长。一线治疗后疾病持续或复发在CD患者中（分别为24%和18%；P <.05）比在ECS患者（分别为15%和15%）和ACS患者（分别为6%和4%）中更常见。自2013年实施专门的患者护理后，2013年以后诊断的CD患者自诊断以来高皮质醇血症的总时间显著短于2013年以前诊断的患者（13.5周对26.1周；P <.0070）。在末次随访（76个月，38 - 163）时，94%的ACS患者、100%的ECS患者和92%的CD患者实现了高皮质醇血症的控制。
大多数不同亚型CS患者可在合理时间内实现生化控制。随着时间推移，高皮质醇血症的控制情况有所改善。
