High mortality within 90 days of diagnosis in patients with Cushing's syndrome: results from the ERCUSYN registry
Por:
Valassi, E, Tabarin, A, Brue, T, Feelders, RA, Reincke, M, Netea-Maier, R, Toth, M, Zacharieva, S, Webb, SM, Tsagarakis, S, Chanson, P, Pfeiffer, M, Droste, M, Komerdus, I, Kastelan, D, Maiter, D, Chabre, O, Franz, H, Santos, A, Strasburger, CJ, Trainer, PJ, Newell-Price, J, Ragnarsson, O, Ambroglo, 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, Demtroder, F, Denes, J, Dimopoulou, C, Dreval, A, Dusek, T, Erdinc, E, Evang, JA, Fazel, J, Fica, S, Ghigo, E, 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, Giraldi, FP, 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, Polovina, TS, 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, ERCUSYN Study Grp
Publicada:
1 nov 2019
Resumen:
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 the commonest cause of death soon after diagnosis, emphasizing the need for careful vigilance at that time, especially in patients presenting with concomitant diabetes mellitus.
Filiaciones:
Valassi, E:
UAB, Hosp St Pau, IIB St Pau, Barcelona, Spain
UAB, Dept Endocrinol Med, Hosp St Pau, Barcelona, Spain
ISCIII, Ctr Invest Biom Red Enfermedades Raras CIBER ER, Barcelona, Spain
Tabarin, A:
Univ Bordeaux, Dept Endocrinol Diabet & Nutr, Bordeaux, France
Brue, T:
Aix Marseille Univ, INSERM U1251, Marseille Med Genet, Marseille, France
Hop Conception, AP HM, Marseille, France
Feelders, RA:
Erasmus MC, Rotterdam, Netherlands
Reincke, M:
Ludwig Maximilians Univ Munchen, Klinikum Univ Munchen, Med Klin & Poliklin 4, Campus Innestadt, Munich, Germany
Netea-Maier, R:
Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands
Toth, M:
Semmelweis Univ, Dept Med 2, Budapest, Hungary
State Hlth Ctr, Dept Med 2, Div Endocrinol, Budapest, Hungary
Zacharieva, S:
Med Univ Sofia, Sofia, Bulgaria
Webb, SM:
UAB, Hosp St Pau, IIB St Pau, Barcelona, Spain
UAB, Dept Endocrinol Med, Hosp St Pau, Barcelona, Spain
ISCIII, Ctr Invest Biom Red Enfermedades Raras CIBER ER, Barcelona, Spain
Tsagarakis, S:
Athens Polyclin Gen Hosp, Athens, Greece
Evangelismos Med Ctr, Athens, Greece
Chanson, P:
Univ Paris Saclay, Univ Paris Sud, UMR S1185, Paris, France
Hop Bicetre, AP HP, Serv Endocrinol & Malad Reprod, Paris, France
INSERM U1185, Paris, France
Pfeiffer, M:
Klinicni Ctr, Ljubljana, Slovenia
Droste, M:
Praxis Endokrinol Droste, Oldenburg, Germany
Komerdus, I:
Moscow Reg Res Clin Inst Na Vladimirsky, Moscow, Russia
Kastelan, D:
Univ Hosp Zagreb, Dept Endocrinol, Zagreb, Croatia
Maiter, D:
UCL Clin Univ St Luc, Brussels, Belgium
Chabre, O:
Hosp Univ, Grenoble, France
Franz, H:
Lohmann & Birkner Hlth Care Consulting GmbH, Berlin, Germany
Santos, A:
UAB, Hosp St Pau, IIB St Pau, Barcelona, Spain
UAB, Dept Endocrinol Med, Hosp St Pau, Barcelona, Spain
ISCIII, Ctr Invest Biom Red Enfermedades Raras CIBER ER, Barcelona, Spain
Strasburger, CJ:
Charit Univ Med, Dept Med CCM, Div Clin Endocrinol, Berlin, Germany
Trainer, PJ:
Christie Hosp, Dept Endocrinol, Manchester, Lancs, England
Newell-Price, J:
Univ Sheffield, Acad Unit Diabet, Med Sch, Dept Oncol & Metab, Sheffield, S Yorkshire, England
Univ Sheffield, Sheffield, S Yorkshire, England
Ragnarsson, O:
Univ Gothenburg, Sahlgrenska Acad, Inst Med, Gothenburg, Sweden
Sahlgrens Univ Hosp, Dept Endocrinol, Gothenburg, Sweden
Gothenburg Univ, Gothenburg, Sweden
Ambroglo, A:
Univ Milan, IRCCS, Ist Auxol Italiano, Milan, Italy
Aranda, G:
UB, Dept Endocrinol, Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Arosio, M:
Univ Milan, Dept Clin Sci & Community Hlth, Unit Endocrine Dis & Diabetol, Milan, Italy
Balomenaki, M:
Evangelismos Med Ctr, Athens Polyclin Gen Hosp, Athens, Greece
Beck-Peccoz, P:
Univ Milan, Fdn IRCCS CaGranda, Endocrinol & Diabetol Unit, Osped Maggiore Policlin, Milan, Italy
Berr-Kirmair, C:
Klinikum Univ Munchen, Med Klin & Poliklin 4, Campus Innestadt, Munich, Germany
Bolanowski, M:
Wroclaw Med Univ, Wroclaw, Poland
Bollerslev, J:
Univ Oslo, Oslo Univ Hosp, Sect Specialized Endocrinol, Oslo, Norway
Univ Oslo, Fac Med, Oslo, Norway
Thierry, B:
Assoc Dev Rech Biol & Med, Marseille, France
Carvalho, D:
Hosp San Joao, Porto, Portugal
Cavagnini, F:
IRCCS, Ist Auxol Italiano, Milan, Italy
Christ, E:
Univ Hosp Bern, Div Endocrinol Diabetol & Clin Nutr, Inselspital, Bern, Switzerland
Demtroder, F:
Rheumatol Dr Demtroder & Kollegen MVZ Dr Eberhard, Zentrum Endokrinol Diabetol, Dortmund, Germany
Denes, J:
State Hlth Ctr, Dept Med 2, Div Endocrinol, Budapest, Hungary
Dimopoulou, C:
Max Planck Gessellschaft Forderung Wissensch eV, Munich, Germany
Dreval, A:
Moscow Reg Res Clin Inst Na Vladimirsky, Moscow, Russia
Dusek, T:
Univ Zagreb, Sch Med, Dept Endocrinol, Univ Hosp Zagreb, Zagreb 10000, Croatia
Erdinc, E:
Uludag Univ, Sch Med, Bursa, Turkey
Evang, JA:
Univ Oslo, Oslo Univ Hosp, Sect Specialized Endocrinol, Oslo, Norway
Univ Oslo, Fac Med, Oslo, Norway
Fazel, J:
Klinikum Univ Munchen, Med Klin & Poliklin 4, Campus Innestadt, Munich, Germany
Fica, S:
Elias Hosp, Bucharest, Romania
Ghigo, E:
Molinette Mauriziano Hosp, Dept Internal Med, Turin, Italy
Greenman, Y:
Inst Endocrinol Metab & Hypertens, Tel Aviv, Israel
Greisa, V:
Med Univ Wien, Vienna, Austria
Halperin, I:
UB, Dept Endocrinol, Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Hanzu, FA:
UB, Dept Endocrinol, Hosp Clin Barcelona, IDIBAPS, Barcelona, Spain
Hermus, A:
Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands
Johannsson, G:
Gothenburg Univ, Gothenburg, Sweden
Kamenicky, P:
Univ Paris Saclay, Univ Paris Sud, UMR S1185, Paris, France
Hop Bicetre, AP HP, Serv Endocrinol & Malad Reprod, Paris, France
INSERM U1185, Paris, France
Kasperlik-Zaluska, A:
Ctr Postgrad Med Educ, Warsaw, Poland
Kirchner, J:
Charite, Dept Med CCM, Div Clin Endocrinol, Berlin, Germany
Darko, K:
Univ Hosp Zagreb, Dept Endocrinol, Zagreb, Croatia
Kraljevic, I:
Univ Hosp Zagreb, Dept Endocrinol, Zagreb, Croatia
Kruszynska, A:
Ctr Postgrad Med Educ, Warsaw, Poland
Lambrescu, I:
Elias Hosp, Bucharest, Romania
Lang, S:
Med Univ Wien, Vienna, Austria
Luger, A:
Max Planck Gessellschaft Forderung Wissensch eV, Munich, Germany
Marpole, N:
NHS Trust, Christie Hosp, Manchester, Lancs, England
Martin, S:
Elias Hosp, Bucharest, Romania
Martinie, M:
Serv Endocrinol Diabetol Nutr, Grenoble, France
Moros, O:
Rheumatol Dr Demtroder & Kollegen MVZ Dr Eberhard, Zentrum Endokrinol Diabetol, Dortmund, Germany
Newell-Price, J:
Univ Sheffield, Acad Unit Diabet, Med Sch, Dept Oncol & Metab, Sheffield, S Yorkshire, England
Univ Sheffield, Sheffield, S Yorkshire, England
Orbetzova, M:
Med Univ, Sv Georgy Univ Hosp, Clin Endocrinol & Metab Dis, Plovdiv, Bulgaria
Paiva, I:
Hosp Univ Coimbra, Coimbra, Portugal
Giraldi, FP:
Univ Milan, IRCCS, Ist Auxol Italiano, Milan, Italy
Pereira, AM:
Leiden Univ, Med Ctr, Leiden, Netherlands
Pickel, J:
Max Planck Gessellschaft Forderung Wissensch eV, Munich, Germany
Pirags, V:
Univ Latvia, Pauls Stradins Clin Univ Hosp, Riga, Latvia
Ragnarsson, O:
Univ Gothenburg, Sahlgrenska Acad, Inst Med, Gothenburg, Sweden
Sahlgrens Univ Hosp, Dept Endocrinol, Gothenburg, Sweden
Gothenburg Univ, Gothenburg, Sweden
Reghina, AD:
Elias Hosp, Bucharest, Romania
Riesgo, P:
Hosp Univ Ribera, Neurosurg Dept, Aizira, Spain
Roberts, M:
NHS Trust, Christie Hosp, Manchester, Lancs, England
Roerink, S:
Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands
Roig, O:
UAB, Hosp St Pau, IIB St Pau, Barcelona, Spain
UAB, Dept Endocrinol Med, Hosp St Pau, Barcelona, Spain
ISCIII, Ctr Invest Biom Red Enfermedades Raras CIBER ER, Barcelona, Spain
Rowan, C:
NHS Trust, Christie Hosp, Manchester, Lancs, England
Rudenko, P:
Estonian Endocrine Soc, Tallinn, Estonia
Sahnoun, MA:
Aix Marseille Univ, CNRS, CRN2M UMR 7286, F-13344 Marseille 15, France
Salvador, J:
Univ Navarra, Pamplona, Spain
Sigurjonsdottir, HA:
Landspitali Univ Hosp, Reykjavik, Iceland
Univ Iceland, Fac Med, Reykjavik, Iceland
Polovina, TS:
Univ Hosp Zagreb, Dept Endocrinol, Zagreb, Croatia
Smith, R:
Oxford Radcliffe Hosp NHS Trust, Oxford, England
Stachowska, B:
Wroclaw Med Univ, Dept Endocrinol Diabetol & Isotope Therapy, Wroclaw, Poland
Stalla, G:
Max Planck Gessellschaft Forderung Wissensch eV, Munich, Germany
Toke, J:
Semmelweis Univ, Dept Med 2, Budapest, Hungary
Ubina, E:
State Hlth Ctr, Dept Med 2, Div Endocrinol, Budapest, Hungary
Vinay, S:
NHS Trust, Christie Hosp, Manchester, Lancs, England
Wagenmakers, M:
Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands
Werner, S:
Praxis Endokrinol Droste, Oldenburg, Germany
Young, J:
Univ Paris Saclay, Univ Paris Sud, UMR S1185, Paris, France
Hop Bicetre, AP HP, Serv Endocrinol & Malad Reprod, Paris, France
INSERM U1185, Paris, France
Zdunowski, P:
Hop Conception, AP HM, Marseille, France
Ctr Postgrad Med Educ, Warsaw, Poland
Zopf, K:
Charite, Dept Med CCM, Div Clin Endocrinol, Berlin, Germany
Zopp, S:
Klinikum Univ Munchen, Med Klin & Poliklin 4, Campus Innestadt, Munich, Germany
Zosin, I:
Romanian Soc Endocrinol, Timisoara, Romania
Green Published, Bronze
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