High mortality within 90 days of diagnosis in patients with Cushing's syndrome: Results from the ERCUSYN Registry

Elena Valassi, Antoine Tabarin, Thierry Brue, Richard A. Feelders, Martin Reincke, Romana Netea-Maier, Miklós Tóth, Sabina Zacharieva, Susan M. Webb, Stylianos Tsagarakis, Philippe Chanson, Marija Pfeifer, Michael Droste, Irina Komerdus, Darko Kastelan, Dominique Maiter, Olivier Chabre, Holger Franz, Alicia Santos, Christian J. StrasburgerPeter J. Trainer, J. Newell-Price, O. Ragnarsson, A. Ambrogio, G. Aranda, M. Arosio, M. Balomenaki, P. Beck-Peccoz, C. Berr-Kirmair, M. Bolanowski, J. Bollerslev, Brue Thierry, D. Carvalho, F. Cavagnini, E. Christ, F. Demtröder, J. Denes, C. Dimopoulou, A. Dreval, T. Dusek, E. Erdinc, J. A. Evang, J. Fazel, S. Fica, E. Ghigo, M. Goth, Y. Greenman, V. Greisa, I. Lambrescu, F. Pecori Giraldi

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)461-472
Number of pages12
JournalEuropean Journal of Endocrinology
Volume181
Issue number5
DOIs
Publication statusPublished - Jan 1 2019

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Cushing Syndrome
Registries
Mortality
Cause of Death
Pituitary ACTH Hypersecretion
Communicable Diseases
Diabetes Mellitus
Cerebrovascular Disorders
Muscle Weakness
Infection
Pulmonary Embolism
Survivors
Disease Progression
Cohort Studies
Cardiovascular Diseases
Therapeutics
Regression Analysis

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

High mortality within 90 days of diagnosis in patients with Cushing's syndrome : Results from the ERCUSYN Registry. / Valassi, Elena; Tabarin, Antoine; Brue, Thierry; Feelders, Richard A.; Reincke, Martin; Netea-Maier, Romana; Tóth, Miklós; Zacharieva, Sabina; Webb, Susan M.; Tsagarakis, Stylianos; Chanson, Philippe; Pfeifer, Marija; Droste, Michael; Komerdus, Irina; Kastelan, Darko; Maiter, Dominique; Chabre, Olivier; Franz, Holger; Santos, Alicia; Strasburger, Christian J.; Trainer, Peter J.; Newell-Price, J.; Ragnarsson, O.; 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, 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.; Lambrescu, I.; Pecori Giraldi, F.

In: European Journal of Endocrinology, Vol. 181, No. 5, 01.01.2019, p. 461-472.

Research output: Contribution to journalArticle

Valassi, E, Tabarin, A, Brue, T, Feelders, RA, Reincke, M, Netea-Maier, R, Tóth, M, Zacharieva, S, Webb, SM, Tsagarakis, S, Chanson, P, Pfeifer, M, Droste, M, Komerdus, I, Kastelan, D, Maiter, D, Chabre, O, Franz, H, Santos, A, Strasburger, CJ, Trainer, PJ, Newell-Price, J, Ragnarsson, O, Ambrogio, A, Aranda, G, Arosio, M, Balomenaki, M, Beck-Peccoz, P, Berr-Kirmair, C, Bolanowski, M, Bollerslev, J, Thierry, B, Carvalho, D, Cavagnini, F, Christ, E, Demtröder, F, Denes, J, Dimopoulou, C, Dreval, A, Dusek, T, Erdinc, E, Evang, JA, Fazel, J, Fica, S, Ghigo, E, Goth, M, Greenman, Y, Greisa, V, Lambrescu, I & Pecori Giraldi, F 2019, 'High mortality within 90 days of diagnosis in patients with Cushing's syndrome: Results from the ERCUSYN Registry', European Journal of Endocrinology, vol. 181, no. 5, pp. 461-472. https://doi.org/10.1530/EJE-19-0464
Valassi, Elena ; Tabarin, Antoine ; Brue, Thierry ; Feelders, Richard A. ; Reincke, Martin ; Netea-Maier, Romana ; Tóth, Miklós ; Zacharieva, Sabina ; Webb, Susan M. ; Tsagarakis, Stylianos ; Chanson, Philippe ; Pfeifer, Marija ; Droste, Michael ; Komerdus, Irina ; Kastelan, Darko ; Maiter, Dominique ; Chabre, Olivier ; Franz, Holger ; Santos, Alicia ; Strasburger, Christian J. ; Trainer, Peter J. ; Newell-Price, J. ; Ragnarsson, O. ; 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, 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. ; Lambrescu, I. ; Pecori Giraldi, F. / High mortality within 90 days of diagnosis in patients with Cushing's syndrome : Results from the ERCUSYN Registry. In: European Journal of Endocrinology. 2019 ; Vol. 181, No. 5. pp. 461-472.
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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.",
author = "Elena Valassi and Antoine Tabarin and Thierry Brue and Feelders, {Richard A.} and Martin Reincke and Romana Netea-Maier and Mikl{\'o}s T{\'o}th and Sabina Zacharieva and Webb, {Susan M.} and Stylianos Tsagarakis and Philippe Chanson and Marija Pfeifer and Michael Droste and Irina Komerdus and Darko Kastelan and Dominique Maiter and Olivier Chabre and Holger Franz and Alicia Santos and Strasburger, {Christian J.} and Trainer, {Peter J.} and J. Newell-Price and O. Ragnarsson and A. Ambrogio and G. Aranda and M. Arosio and M. Balomenaki and P. Beck-Peccoz and C. Berr-Kirmair and M. Bolanowski and J. Bollerslev and Brue Thierry and D. Carvalho and F. Cavagnini and E. Christ and F. Demtr{\"o}der and J. Denes and C. Dimopoulou and A. Dreval and T. Dusek and E. Erdinc and Evang, {J. A.} and J. Fazel and S. Fica and E. Ghigo and M. Goth and Y. Greenman and V. Greisa and I. Lambrescu and {Pecori Giraldi}, F.",
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language = "English",
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TY - JOUR

T1 - High mortality within 90 days of diagnosis in patients with Cushing's syndrome

T2 - Results from the ERCUSYN Registry

AU - Valassi, Elena

AU - Tabarin, Antoine

AU - Brue, Thierry

AU - Feelders, Richard A.

AU - Reincke, Martin

AU - Netea-Maier, Romana

AU - Tóth, Miklós

AU - Zacharieva, Sabina

AU - Webb, Susan M.

AU - Tsagarakis, Stylianos

AU - Chanson, Philippe

AU - Pfeifer, Marija

AU - Droste, Michael

AU - Komerdus, Irina

AU - Kastelan, Darko

AU - Maiter, Dominique

AU - Chabre, Olivier

AU - Franz, Holger

AU - Santos, Alicia

AU - Strasburger, Christian J.

AU - Trainer, Peter J.

AU - Newell-Price, J.

AU - Ragnarsson, O.

AU - Ambrogio, A.

AU - Aranda, G.

AU - Arosio, M.

AU - Balomenaki, M.

AU - Beck-Peccoz, P.

AU - Berr-Kirmair, C.

AU - Bolanowski, M.

AU - Bollerslev, J.

AU - Thierry, Brue

AU - Carvalho, D.

AU - Cavagnini, F.

AU - Christ, E.

AU - Demtröder, F.

AU - Denes, J.

AU - Dimopoulou, C.

AU - Dreval, A.

AU - Dusek, T.

AU - Erdinc, E.

AU - Evang, J. A.

AU - Fazel, J.

AU - Fica, S.

AU - Ghigo, E.

AU - Goth, M.

AU - Greenman, Y.

AU - Greisa, V.

AU - Lambrescu, I.

AU - Pecori Giraldi, F.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

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