Cited by 134Pituitary Gland Disorders and TreatmentsAdrenal and Paraganglionic TumorsNeuroendocrine Tumor Research Advances

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Overall and Disease-Specific Mortality in Patients With Cushing Disease: A Swedish Nationwide Study

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Abstract

CONTEXT: Whether patients with Cushing disease (CD) in remission have increased mortality is still debatable. OBJECTIVE: To study overall and disease-specific mortality and predictive factors in an unselected nationwide cohort of patients with CD. DESIGN, PATIENTS, AND METHODS: A retrospective study of patients diagnosed with CD, identified in the Swedish National Patient Registry between 1987 and 2013. Medical records were systematically reviewed to verify the diagnosis. Standardized mortality ratios (SMRs) with 95% CIs were calculated and Cox regression models were used to identify predictors of mortality. RESULTS: Of 502 identified patients with CD (n = 387 women; 77%), 419 (83%) were confirmed to be in remission. Mean age at diagnosis was 43 (SD, 16) years and median follow-up was 13 (interquartile range, 6 to 23) years. The observed number of deaths was 133 vs 54 expected, resulting in an overall SMR of 2.5 (95% CI, 2.1 to 2.9). The commonest cause of death was cardiovascular diseases (SMR, 3.3; 95% CI, 2.6 to 4.3). Excess mortality was also found associated with infections and suicide. For patients in remission, the SMR was 1.9 (95% CI, 1.5 to 2.3); bilateral adrenalectomy and glucocorticoid replacement therapy were independently associated with increased mortality, whereas GH replacement was associated with improved outcome. CONCLUSION: Findings from this large nationwide study indicate that patients with CD have excess mortality. The findings illustrate the importance of achieving remission and continued active surveillance, along with adequate hormone replacement and evaluation of cardiovascular risk and mental health.

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Research works printed but not linked: 27

  1. Plotz, 1952, The natural history of Cushing’s syndrome, Am J Med, 13, 597, 10.1016/0002-9343(52)90027-2 · DOI
  2. Nieman, 2015, Treatment of Cushing’s syndrome: an Endocrine Society Clinical practice guideline, J Clin Endocrinol Metab, 100, 2807, 10.1210/jc.2015-1818 · DOI · Paper page
  3. Lindholm, 2001, Incidence and late prognosis of Cushing’s syndrome: a population-based study, J Clin Endocrinol Metab, 86, 117
  4. Hammer, 2004, Transsphenoidal microsurgery for Cushing’s disease: initial outcome and long-term results, J Clin Endocrinol Metab, 89, 6348, 10.1210/jc.2003-032180 · DOI
  5. Clayton, 2011, Mortality and morbidity in Cushing’s disease over 50 years in Stoke-on-Trent, UK: audit and meta-analysis of literature, J Clin Endocrinol Metab, 96, 632, 10.1210/jc.2010-1942 · DOI
  6. Dekkers, 2007, Mortality in patients treated for Cushing’s disease is increased, compared with patients treated for nonfunctioning pituitary macroadenoma, J Clin Endocrinol Metab, 92, 976, 10.1210/jc.2006-2112 · DOI
  7. Tjörnstrand, 2014, The incidence rate of pituitary adenomas in western Sweden for the period 2001-2011, Eur J Endocrinol, 171, 519, 10.1530/EJE-14-0144 · DOI
  8. Etxabe, 1994, Morbidity and mortality in Cushing’s disease: an epidemiological approach, Clin Endocrinol (Oxf), 40, 479, 10.1111/j.1365-2265.1994.tb02486.x · DOI · Paper page
  9. Pikkarainen, 1999, The survival and well-being of patients treated for Cushing’s syndrome, J Intern Med, 245, 463, 10.1046/j.1365-2796.1999.00483.x · DOI
  10. Lambert, 2013, Predictors of mortality and long-term outcomes in treated Cushing’s disease: a study of 346 patients, J Clin Endocrinol Metab, 98, 1022, 10.1210/jc.2012-2893 · DOI
  11. Brooke, 2017, The Swedish cause of death register, Eur J Epidemiol, 32, 765, 10.1007/s10654-017-0316-1 · DOI
  12. Altman, 2003, Interaction revisited: the difference between two estimates, BMJ, 326, 219, 10.1136/bmj.326.7382.219 · DOI · Paper page
  13. Dorn, 1997, The longitudinal course of psychopathology in Cushing’s syndrome after correction of hypercortisolism, J Clin Endocrinol Metab, 82, 912
  14. Sonino, 1998, Clinical correlates of major depression in Cushing’s disease, Psychopathology, 31, 302, 10.1159/000029054 · DOI
  15. Santos, 2017, Psychiatric symptoms in patients with Cushing’s syndrome: prevalence, diagnosis and management, Drugs, 77, 829, 10.1007/s40265-017-0735-z · DOI
  16. Pivonello, 2016, Complications of Cushing’s syndrome: state of the art, Lancet Diabetes Endocrinol, 4, 611, 10.1016/S2213-8587(16)00086-3 · DOI
  17. Keil, 2016, Cases of psychiatric morbidity in pediatric patients after remission of Cushing syndrome, Pediatrics, 137, e1, 10.1542/peds.2015-2234 · DOI
  18. Zeiger, 1993, Effective reversibility of the signs and symptoms of hypercortisolism by bilateral adrenalectomy, Surgery, 114, 1138
  19. Välimäki, 1984, Long-term results of adrenal surgery in patients with Cushing’s syndrome due to adrenocortical adenoma, Clin Endocrinol (Oxf), 20, 229, 10.1111/j.1365-2265.1984.tb00078.x · DOI
  20. Hammarstrand, 2017, Higher glucocorticoid replacement doses are associated with increased mortality in patients with pituitary adenoma, Eur J Endocrinol, 177, 251, 10.1530/EJE-17-0340 · DOI · Paper page
  21. Zueger, 2012, Glucocorticoid replacement and mortality in patients with nonfunctioning pituitary adenoma, J Clin Endocrinol Metab, 97, E1938, 10.1210/jc.2012-2432 · DOI · Paper page
  22. O’Reilly, 2016, ACTH and gonadotropin deficiencies predict mortality in patients treated for nonfunctioning pituitary adenoma: long-term follow-up of 519 patients in two large European centres, Clin Endocrinol (Oxf), 85, 748, 10.1111/cen.13141 · DOI
  23. Sherlock, 2009, ACTH deficiency, higher doses of hydrocortisone replacement, and radiotherapy are independent predictors of mortality in patients with acromegaly, J Clin Endocrinol Metab, 94, 4216, 10.1210/jc.2009-1097 · DOI · Paper page
  24. Olsson, 2017, Life expectancy in patients with pituitary adenoma receiving growth hormone replacement, Eur J Endocrinol, 176, 67, 10.1530/EJE-16-0450 · DOI
  25. Olsson, 2017, Time trends of mortality in patients with non-functioning pituitary adenoma: a Swedish nationwide study, Pituitary, 20, 218, 10.1007/s11102-016-0764-8 · DOI
  26. Esposito, 2018, Decreasing mortality and changes in treatment patterns in patients with acromegaly from a nationwide study, Eur J Endocrinol, 178, 459, 10.1530/EJE-18-0015 · DOI
  27. Ragnarsson, 2013, Cushing’s syndrome: a structured short- and long-term management plan for patients in remission, Eur J Endocrinol, 169, R139, 10.1530/EJE-13-0534 · DOI

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Metadata

Authors
Ragnarsson, O., Olsson, D. S., Papakokkinou, E., Chantzichristos, D., Dahlqvist, P., Segerstedt, E., Olsson, T., Petersson, M., Berinder, K., Bensing, S., Höybye, C., Edén-Engström, B., Burman, P., Bonelli, L., Follin, C., Petranek, D., Erfurth, E. M., Wahlberg, J., Ekman, B., Åkerman, A.-K., Schwarcz, E., Bryngelsson, I.-L., & Johannsson, G.
Affiliations
  • Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, Göteborg, Sweden
  • Sahlgrenska University Hospital (SE)
  • Umeå University (SE)
  • Karolinska University Hospital (SE)
  • Department of Medical Sciences; Endocrinology, Diabetes and Metabolism, Uppsala University Hospital, Uppsala, Sweden
  • Skåne University Hospital (SE)
  • Linköping University (SE)
  • Örebro University (SE)
  • Örebro University Hospital (SE)
Venue
The Journal of Clinical Endocrinology & Metabolism
Published
2019-02-01
Volume
104
Issue
6
Pages
2375-2384
ISSN
  • 0021-972X (ISSN-L)
  • 1945-7197
OpenAlex
W2914799087
Cited by
134
References
36
Citations by year
2026: 17 · 2025: 17 · 2024: 14 · 2023: 18 · 2022: 29 · 2021: 18 · 2020: 14 · 2019: 7
FWCI
13.84
Type
journal article
Language
en
Open access
Locations
  • The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Göteborgs Läkaresällskap
  • Västra Götalandsregionen
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Oncology › Neuroendocrine Tumor Research Advances
Keywords
Cushing's disease, mortality, remission, cardiovascular disease, suicide, infection, bilateral adrenalectomy, glucocorticoid replacement therapy, growth hormone replacement therapy, hormone replacement therapy, mortality risk factors, active surveillance
MeSH terms
Adult, Cardiovascular Diseases, Female, Humans, Male, Middle Aged, Retrospective Studies, Proportional Hazards Models, Hormone Replacement Therapy, Pituitary ACTH Hypersecretion

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