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. Strasburger; Peter J. Trainer; John Newell-Price; Oskar 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. Halperin; F. A. Hanzu; A. Hermus; G. Johannsson; P. Kamenicky; A. Kasperlik-Zaluska; J. Kirchner; Kastelan Darko; I. Kraljevic; A. Kruszynska; I. Lambrescu; S. Lang; A. Luger; N. Marpole; S. Martin; M. Martinie; O. Moros; J. Newell-Price; M. Orbetzova; I. Paiva; F. Pecori Giraldi; A. M. Pereira; J. Pickel; V. Pirags; O. Ragnarsson; A. D. Reghina; P. Riesgo; M. Roberts; S. Roerink; O. Roig; C. Rowan; P. Rudenko; M. A. Sahnoun; J. Salvador; H. A. Sigurjonsdottir; T. Skoric Polovina; R. Smith; B. Stachowska; G. Stalla; J. Toke; E. Ubina; S. Vinay; M. Wagenmakers; S. Werner; J. Young; P. Zdunowski; K. Zopf; S. Zopp; I. Zosin

doi:10.1530/EJE-19-0464

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, John Newell-Price, Oskar 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. Halperin, F. A. Hanzu, A. Hermus, G. Johannsson, P. Kamenicky, A. Kasperlik-Zaluska, J. Kirchner, Kastelan Darko, I. Kraljevic, A. Kruszynska, I. Lambrescu, S. Lang, A. Luger, N. Marpole, S. Martin, M. Martinie, O. Moros, J. Newell-Price, M. Orbetzova, I. Paiva, F. Pecori Giraldi, A. M. Pereira, J. Pickel, V. Pirags, O. Ragnarsson, A. D. Reghina, P. Riesgo, M. Roberts, S. Roerink, O. Roig, C. Rowan, P. Rudenko, M. A. Sahnoun, J. Salvador, H. A. Sigurjonsdottir, T. Skoric Polovina, R. Smith, B. Stachowska, G. Stalla, J. Toke, E. Ubina, S. Vinay, M. Wagenmakers, S. Werner, J. Young, P. Zdunowski, K. Zopf, S. Zopp, I. Zosin

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

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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.

Original language	English
Pages (from-to)	461-472
Number of pages	12
Journal	European Journal of Endocrinology
Volume	181
Issue number	5
DOIs	https://doi.org/10.1530/EJE-19-0464
State	Published - 2019
Externally published	Yes

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10.1530/EJE-19-0464

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Valassi, E., Tabarin, A., Brue, T., Feelders, R. A., Reincke, M., Netea-Maier, R., Tóth, M., Zacharieva, S., Webb, S. M., Tsagarakis, S., Chanson, P., Pfeifer, M., Droste, M., Komerdus, I., Kastelan, D., Maiter, D., Chabre, O., Franz, H., Santos, A., ... Zosin, I. (2019). High mortality within 90 days of diagnosis in patients with Cushing's syndrome: Results from the ERCUSYN Registry. European Journal of Endocrinology, 181(5), 461-472. https://doi.org/10.1530/EJE-19-0464

@article{94312456c887453081074a0a9554da85,

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

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 John Newell-Price and Oskar 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. Halperin and Hanzu, {F. A.} and A. Hermus and G. Johannsson and P. Kamenicky and A. Kasperlik-Zaluska and J. Kirchner and Kastelan Darko and I. Kraljevic and A. Kruszynska and I. Lambrescu and S. Lang and A. Luger and N. Marpole and S. Martin and M. Martinie and O. Moros and J. Newell-Price and M. Orbetzova and I. Paiva and {Pecori Giraldi}, F. and Pereira, {A. M.} and J. Pickel and V. Pirags and O. Ragnarsson and Reghina, {A. D.} and P. Riesgo and M. Roberts and S. Roerink and O. Roig and C. Rowan and P. Rudenko and Sahnoun, {M. A.} and J. Salvador and Sigurjonsdottir, {H. A.} and {Skoric Polovina}, T. and R. Smith and B. Stachowska and G. Stalla and J. Toke and E. Ubina and S. Vinay and M. Wagenmakers and S. Werner and J. Young and P. Zdunowski and K. Zopf and S. Zopp and I. Zosin",

note = "Publisher Copyright: {\textcopyright} 2019 European Society of Endocrinology Printed in Great Britain.",

year = "2019",

doi = "10.1530/EJE-19-0464",

language = "אנגלית",

volume = "181",

pages = "461--472",

journal = "European Journal of Endocrinology",

issn = "0804-4643",

publisher = "BioScientifica Ltd.",

number = "5",

}

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, Halperin, I, Hanzu, FA, Hermus, A, Johannsson, G, Kamenicky, P, Kasperlik-Zaluska, A, Kirchner, J, Darko, K, 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, Toke, J, Ubina, E, Vinay, S, Wagenmakers, M, Werner, S, Young, J, Zdunowski, P, Zopf, K, Zopp, S & Zosin, I 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

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, John

AU - Ragnarsson, Oskar

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 - Halperin, I.

AU - Hanzu, F. A.

AU - Hermus, A.

AU - Johannsson, G.

AU - Kamenicky, P.

AU - Kasperlik-Zaluska, A.

AU - Kirchner, J.

AU - Darko, Kastelan

AU - Kraljevic, I.

AU - Kruszynska, A.

AU - Lambrescu, I.

AU - Lang, S.

AU - Luger, A.

AU - Marpole, N.

AU - Martin, S.

AU - Martinie, M.

AU - Moros, O.

AU - Newell-Price, J.

AU - Orbetzova, M.

AU - Paiva, I.

AU - Pecori Giraldi, F.

AU - Pereira, A. M.

AU - Pickel, J.

AU - Pirags, V.

AU - Ragnarsson, O.

AU - Reghina, A. D.

AU - Riesgo, P.

AU - Roberts, M.

AU - Roerink, S.

AU - Roig, O.

AU - Rowan, C.

AU - Rudenko, P.

AU - Sahnoun, M. A.

AU - Salvador, J.

AU - Sigurjonsdottir, H. A.

AU - Skoric Polovina, T.

AU - Smith, R.

AU - Stachowska, B.

AU - Stalla, G.

AU - Toke, J.

AU - Ubina, E.

AU - Vinay, S.

AU - Wagenmakers, M.

AU - Werner, S.

AU - Young, J.

AU - Zdunowski, P.

AU - Zopf, K.

AU - Zopp, S.

AU - Zosin, I.

PY - 2019

Y1 - 2019

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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U2 - 10.1530/EJE-19-0464

DO - 10.1530/EJE-19-0464

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C2 - 31480014

AN - SCOPUS:85072950611

SN - 0804-4643

VL - 181

SP - 461

EP - 472

JO - European Journal of Endocrinology

JF - European Journal of Endocrinology

IS - 5

ER -

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

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