High mortality within 90 days of diagnosis in patients with Cushing's syndrome: results from the ERCUSYN registry.

Autor:	Valassi E; IIB-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., Tabarin A; Department of Endocrinology, Diabetes and Nutrition, University of Bordeaux, Bordeaux, France., Brue T; Aix-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., Feelders RA; Erasmus University Medical Centre, Rotterdam, The Netherlands., Reincke M; Medizinische Klinik und Poliklinik IV, Campus Innestadt, Klinikum der Universität München, Ludwig-Maximilians-Universität München, Munich, Germany., Netea-Maier R; Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands., Tóth M; 2nd Department of Medicine, Semmelweis University, Budapest, Hungary., Zacharieva S; Medical University of Sofia, Sofia, Bulgary., Webb SM; IIB-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., Tsagarakis S; Athens Polyclinic General Hospital & Evangelismos Hospital, Athens, Greece., Chanson P; Univ Paris-Sud, Université Paris-Saclay UMR-S1185, Le Kremlin Bicêtre, Paris, France.; Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service de Endocrinologie et des Maladies de la Reproduction, Le Kremlin Bicêtre, Paris, France.; Institut National de la Santé et de la Recherche Médicale U1185, Le Kremlin Bicêtre, Paris, France., Pfeiffer M; Klinicni Center, Ljubljana, Slovenia., Droste M; Praxis für Endokrinologie Droste, Oldenburg, Germany., Komerdus I; Moscow Regional Research Clinical Institute n.a. Vladimirsky, Moscow, Russia., Kastelan D; Department of Endocrinology, University Hospital Zagreb, Zagreb, Croatia., Maiter D; UCL Cliniques Universitaires St Luc, Brussels, Belgium., Chabre O; Hospitalier Universitaire, Grenoble, France., Franz H; Lohmann & Birkner Health Care Consulting GmbH, Berlin, Germany., Santos A; IIB-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., Strasburger CJ; Division of Clinical Endocrinology, Department of Medicine CCM, Charité-Universitätsmedizin, Berlin, Germany., Trainer PJ; Department of Endocrinology, Christie Hospital, Manchester, UK., Newell-Price J; Academic Unit of Diabetes, Endocrinology and Reproduction, Department of Oncology and Metabolism, The Medical School, University of Sheffield, Sheffield, UK., Ragnarsson O; Institute of Medicine at Sahlgrenska Academy, University of Gothenburg and the Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden.
Jazyk:	angličtina
Zdroj:	European journal of endocrinology [Eur J Endocrinol] 2019 Nov; Vol. 181 (5), pp. 461-472.
DOI:	10.1530/EJE-19-0464
Abstrakt:	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.
Databáze:	MEDLINE
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