High mortality within 90 days of diagnosis in patients with Cushing’s syndrome: results from the ERCUSYN registry
Author:
Valassi Elena1, Tabarin Antoine2, Brue Thierry3, Feelders Richard A4, Reincke Martin5, Netea-Maier Romana6, Tóth Miklós7, Zacharieva Sabina8, Webb Susan M1, Tsagarakis Stylianos9, Chanson Philippe101112, Pfeiffer Marija13, Droste Michael14, Komerdus Irina15, Kastelan Darko16, Maiter Dominique17, Chabre Olivier18, Franz Holger19, Santos Alicia1, Strasburger Christian J20, Trainer Peter J21, Newell-Price John22, Ragnarsson Oskar23, _ _, _ _, Ambrogio A, Aranda G, Arosio M, Balomenaki M, Beck-Peccoz P, Berr-Kirmair C, Bolanowski M., Bollerslev J, Thierry Brue, Carvalho D, Cavagnini F, Christ E, Demtröder Zentrum fur Endokrinologi F, Denes J, Dimopoulou C, Dreval A, Dusek T, Erdinc E, Evang J A, Fazel J, Fica S, Ghigo E, Goth M, Greenman Y, Greisa V, Halperin I, Hanzu FA, Hermus A, Johannsson G, Kamenicky P, Kasperlik-Zaluska A, Kirchner J, Darko Kastelan, Kraljevic I, Kruszynska A, Lambrescu I, Lang S, Luger A, Marpole N, Martin S, Martinie M, Moros O, Newell-Price J, Orbetzova M, Paiva I, Pecori Giraldi F, Pereira AM, Pickel J, Pirags V, Ragnarsson O, Reghina AD, Riesgo P, Roberts M, Roerink S, Roig O, Rowan C, Rudenko P, Sahnoun MA, Salvador J, Sigurjonsdottir HA, Skoric Polovina T, Smith R, Stachowska B, Stalla G, Tőke J, Ubina E, Vinay S, Wagenmakers M, Werner S, Young J, Zdunowski P, Zopf K, Zopp S, Zosin I
Affiliation:
1. 1IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau, UAB, and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain 2. 2Department of Endocrinology, Diabetes and Nutrition, University of Bordeaux, Bordeaux, France 3. 3Aix-Marseille Université, Institut National de la Santé et de la Recherche Médicale INSERM U1251, Marseille Medical Genetics, Marseille, France and Assistance Publique Hôpitaux de Marseille (APHM), Hôpital de la Conception, Marseille, France 4. 4Erasmus University Medical Centre, Rotterdam, The Netherlands 5. 5Medizinische Klinik und Poliklinik IV, Campus Innestadt, Klinikum der Universität München, Ludwig-Maximilians-Universität München, Munich, Germany 6. 6Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands 7. 72nd Department of Medicine, Semmelweis University, Budapest, Hungary 8. 8Medical University of Sofia, Sofia, Bulgary 9. 9Athens Polyclinic General Hospital & Evangelismos Hospital, Athens, Greece 10. 10Univ Paris-Sud, Université Paris-Saclay UMR-S1185, Le Kremlin Bicêtre, Paris, France 11. 11Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service de Endocrinologie et des Maladies de la Reproduction, Le Kremlin Bicêtre, Paris, France 12. 12Institut National de la Santé et de la Recherche Médicale U1185, Le Kremlin Bicêtre, Paris, France 13. 13Klinicni Center, Ljubljana, Slovenia 14. 14Praxis für Endokrinologie Droste, Oldenburg, Germany 15. 15Moscow Regional Research Clinical Institute n.a. Vladimirsky, Moscow, Russia 16. 16Department of Endocrinology, University Hospital Zagreb, Zagreb, Croatia 17. 17UCL Cliniques Universitaires St Luc, Brussels, Belgium 18. 18Hospitalier Universitaire, Grenoble, France 19. 19Lohmann & Birkner Health Care Consulting GmbH, Berlin, Germany 20. 20Division of Clinical Endocrinology, Department of Medicine CCM, Charité-Universitätsmedizin, Berlin, Germany 21. 21Department of Endocrinology, Christie Hospital, Manchester, UK 22. 22Academic Unit of Diabetes, Endocrinology and Reproduction, Department of Oncology and Metabolism, The Medical School, University of Sheffield, Sheffield, UK 23. 23Institute of Medicine at Sahlgrenska Academy, University of Gothenburg and the Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden
Abstract
Objective
Patients with Cushing’s syndrome (CS) have increased mortality. The aim of this study was to evaluate the causes and time of death in a large cohort of patients with CS and to establish factors associated with increased mortality.
Methods
In this cohort study, we analyzed 1564 patients included in the European Registry on CS (ERCUSYN); 1045 (67%) had pituitary-dependent CS, 385 (25%) adrenal-dependent CS, 89 (5%) had an ectopic source and 45 (3%) other causes. The median (IQR) overall follow-up time in ERCUSYN was 2.7 (1.2–5.5) years.
Results
Forty-nine patients had died at the time of the analysis; 23 (47%) with pituitary-dependent CS, 6 (12%) with adrenal-dependent CS, 18 (37%) with ectopic CS and two (4%) with CS due to other causes. Of 42 patients whose cause of death was known, 15 (36%) died due to progression of the underlying disease, 13 (31%) due to infections, 7 (17%) due to cardiovascular or cerebrovascular disease and 2 due to pulmonary embolism. The commonest cause of death in patients with pituitary-dependent CS and adrenal-dependent CS were infectious diseases (n = 8) and progression of the underlying tumor (n = 10) in patients with ectopic CS. Patients who had died were older and more often males, and had more frequently muscle weakness, diabetes mellitus and ectopic CS, compared to survivors. Of 49 deceased patients, 22 (45%) died within 90 days from start of treatment and 5 (10%) before any treatment was given. The commonest cause of deaths in these 27 patients were infections (n = 10; 37%). In a regression analysis, age, ectopic CS and active disease were independently associated with overall death before and within 90 days from the start of treatment.
Conclusion
Mortality rate was highest in patients with ectopic CS. Infectious diseases were the commonest cause of death soon after diagnosis, emphasizing the need for careful clinical vigilance at that time, especially in patients presenting with concomitant diabetes mellitus.
Subject
Endocrinology,General Medicine,Endocrinology, Diabetes and Metabolism
Cited by
62 articles.
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