Patients with Cushing syndrome (CS) have an increased risk of venous thromboembolism (VTE). However, evidence regarding the occurrence of VTE and current thromboprophylaxis strategies in patients with CS remains limited due to the rarity of the condition and the lack of large prospective studies.

To evaluate current clinical management of new cases of Cushing syndrome across Europe, with a specific focus on the occurrence of venous thromboembolism and the use of thromboprophylaxis.

A survey was conducted among healthcare professionals reporting new cases of Cushing syndrome through the electronic reporting tool (e-REC) of the European Registries for Rare Endocrine Conditions (EuRRECa) and the relevant Main Thematic Groups (MTGs) of the European Reference Network on Rare Endocrine Conditions (Endo-ERN). Data on newly diagnosed patients with Cushing syndrome between January 2021 and July 2022 were collected.

A total of 222 patients were included (mean age 44 years, 165 females). Cushing disease was diagnosed in 141 patients (64%), adrenal Cushing syndrome in 69 patients (31%), and ectopic Cushing syndrome in 12 patients (5.4%). Cortisol-lowering medication was initiated in 38% of patients. Thromboprophylaxis was administered to 154 patients (69%), including patients receiving chronic anticoagulant treatment, with low-molecular-weight heparins used in 96% of cases. Venous thromboembolism occurred in six patients (2.7%), including one fatal event. The incidence rate of VTE after Cushing syndrome diagnosis was 14.6 per 1000 person-years (95% CI 5.5–38.6).

Thirty percent of patients with Cushing syndrome did not receive preoperative thromboprophylaxis during active disease, and half of the VTE events occurred during this period despite thromboprophylaxis. Prospective studies are needed to establish optimal strategies for prevention of venous thromboembolism in patients with Cushing syndrome.

This European survey demonstrates considerable variation in the initiation and duration of thromboprophylaxis strategies for patients with Cushing syndrome. The data generated through EuRRECa and Endo-ERN provide important insights into current clinical practice and highlight the need for evidence-based recommendations to reduce thromboembolic complications.