Background Patients with Cushing syndrome (CS) are at increased risk of venous thromboembolism (VTE).Objective The aim was to evaluate the current management of new cases of CS with a focus on VTE and thromboprophylaxis.Design and methods A survey was conducted within those that report in the electronic reporting tool (e-REC) of the European Registries for Rare Endocrine Conditions (EuRRECa) and the involved main thematic groups (MTG's) of the European Reference Networks for Rare Endocrine Disorders (Endo-ERN) on new patients with CS from January 2021 to July 2022.Results Of 222 patients (mean age 44 years, 165 females), 141 patients had Cushing disease (64%), 69 adrenal CS (31%), and 12 patients with ectopic CS (5.4%). The mean follow-up period post-CS diagnosis was 15 months (range 3-30). Cortisol-lowering medications were initiated in 38% of patients. One hundred fifty-four patients (69%) received thromboprophylaxis (including patients on chronic anticoagulant treatment), of which low-molecular-weight heparins were used in 96% of cases. VTE was reported in six patients (2.7%), of which one was fatal: two long before CS diagnosis, two between diagnosis and surgery, and two postoperatively. Three patients were using thromboprophylaxis at time of the VTE diagnosis. The incidence rate of VTE in patients after Cushing syndrome diagnosis in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years.Conclusion Thirty percent of patients with CS did not receive preoperative thromboprophylaxis during their active disease stage, and half of the VTE cases even occurred during this stage despite thromboprophylaxis. Prospective trials to establish the optimal thromboprophylaxis strategy in CS patients are highly needed.Significance statement The incidence rate of venous thromboembolism in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years. Notably, this survey showed that there is great heterogeneity regarding time of initiation and duration of thromboprophylaxis in expert centers throughout Europe.
Venous thromboembolism in Cushing syndrome: results from an EuRRECa and Endo-ERN survey / Cherenko, M; Appelman-Dijkstra, N M; Priego Zurita, A L; Biermasz, N R; Dekkers, O M; Klok, F A; Reisch, N; Aulinas, A; Biagetti, B; Cannavo, S; Canu, L; Detomas, M; Devuyst, F; Falhammar, H; Feelders, R A; Ferrau, F; Gatto, F; Grasselli, C; van Houten, P; Hoybye, C; Isidori, A M; Kyrilli, A; Loli, P; Maiter, D; Nowak, E; Pivonello, R; Ragnarsson, O; Steenaard, R V; Unger, N; van de Ven, A; Webb, S M; Yeste, D; Ahmed, S F; Pereira, A M. - In: ENDOCRINE CONNECTIONS. - ISSN 2049-3614. - 13:6(2024). [10.1530/EC-24-0046]
Venous thromboembolism in Cushing syndrome: results from an EuRRECa and Endo-ERN survey
Isidori, A M;
2024
Abstract
Background Patients with Cushing syndrome (CS) are at increased risk of venous thromboembolism (VTE).Objective The aim was to evaluate the current management of new cases of CS with a focus on VTE and thromboprophylaxis.Design and methods A survey was conducted within those that report in the electronic reporting tool (e-REC) of the European Registries for Rare Endocrine Conditions (EuRRECa) and the involved main thematic groups (MTG's) of the European Reference Networks for Rare Endocrine Disorders (Endo-ERN) on new patients with CS from January 2021 to July 2022.Results Of 222 patients (mean age 44 years, 165 females), 141 patients had Cushing disease (64%), 69 adrenal CS (31%), and 12 patients with ectopic CS (5.4%). The mean follow-up period post-CS diagnosis was 15 months (range 3-30). Cortisol-lowering medications were initiated in 38% of patients. One hundred fifty-four patients (69%) received thromboprophylaxis (including patients on chronic anticoagulant treatment), of which low-molecular-weight heparins were used in 96% of cases. VTE was reported in six patients (2.7%), of which one was fatal: two long before CS diagnosis, two between diagnosis and surgery, and two postoperatively. Three patients were using thromboprophylaxis at time of the VTE diagnosis. The incidence rate of VTE in patients after Cushing syndrome diagnosis in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years.Conclusion Thirty percent of patients with CS did not receive preoperative thromboprophylaxis during their active disease stage, and half of the VTE cases even occurred during this stage despite thromboprophylaxis. Prospective trials to establish the optimal thromboprophylaxis strategy in CS patients are highly needed.Significance statement The incidence rate of venous thromboembolism in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years. Notably, this survey showed that there is great heterogeneity regarding time of initiation and duration of thromboprophylaxis in expert centers throughout Europe.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
