Mortality in Cushing's syndrome: data from 386 patients from a single tertiary referral center
Abstract
Objective: Data on the incidence, mortality, and causes of death in patients with Cushing's syndrome (CS) are scarce, due to the rarity of CS. The aim of the study was to analyze mortality in a large cohort of patients of all etiologies and to determine the cause of death. Design: This was a retrospective study of patients with CS, treated over a period of 45 years in the main tertiary referral center in Bulgaria. Methods: Three hundred and eighty-six patients with CS of all etiologies were included. The main outcome measures were the standardized mortality ratio (SMR) and the cause of death. Results: Mean (±s.d.) age at diagnosis was 38±13 years; 84% of patients were women; mean follow-up was 85 months (range: 0–494 months). The SMR in the CS cohort was 4.05 (95% CI 2.50–5.80) (P<0.0001). The following subgroups did not have a significantly increased SMR: patients with Cushing's disease SMR – 1.88 (95% CI 0.69–4.08), adrenal adenomas 1.67 (95% CI 0.20–6.02), and ACTH-independent bilateral adrenal hyperplasia 1.14 (95% CI 0.21–6.34). Patients with adrenal carcinomas, ectopic CS, and those with CS of undetermined etiology had significantly increased SMR: 48.00 (95% CI 30.75–71.42), 13.33 (95% CI 0.00–24.59), and 4.00 (95% CI 0.48–14.45) respectively (P<0.0001). The significant predictors for mortality were active disease at death, age, male sex, etiology of the disease, and the overall duration of active disease. The major causes of death were vascular events (40%) – cardiovascular 29%, and cerebrovascular 11% – followed by infections (12%). Conclusions: Patients with CS have increased mortality due to vascular events and infections.
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Hypercortisolism is associated with 3.5 to 5 times increased mortality risk compared with the general population.
“Hypercortisolism is associated with an increase in cardiovascular events (myocardial infarction), cerebrovascular events (stroke), sepsis, and thromboembolism with 3.5 to 5 times increased mortality risk compared with the general population (7-11).”
From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [11]
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- Authors
- Yaneva, M., Kalinov, K., & Zacharieva, S.
- Affiliations
- Medical University of Sofia (BG)
- New Bulgarian University (BG)
- Venue
- European Journal of Endocrinology
- Publisher
- Oxford University Press (OUP)
- Published
- 2013-11
- Volume
- 169
- Issue
- 5
- Pages
- 621-627
- ISSN
- 0804-4643 (ISSN-L)
- 1479-683X
- OpenAlex
- W2122999501
- Cited by
- 93
- References
- 17
- Citations by year
- 2026: 4 · 2025: 10 · 2024: 8 · 2023: 4 · 2022: 8 · 2021: 9 · 2020: 5 · 2019: 5 · 2018: 14 · 2016: 9 · 2015: 8 · 2014: 9
- FWCI
- 6.81
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Journal of Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Keywords
- Cushing's syndrome, mortality, standardized mortality ratio, causes of death, Cushing's disease, adrenal adenoma, ACTH-independent Cushing's syndrome, ectopic ACTH syndrome, adrenocortical carcinoma, vascular events, cardiovascular mortality, cerebrovascular disease mortality, infection, mortality risk factors
- MeSH terms
- Adenoma, Adolescent, Adrenal Gland Neoplasms, Adrenal Hyperplasia, Congenital, Adult, Aged, Aged, 80 and over, Bulgaria, Carcinoma, Cause of Death, Child, Child, Preschool, Cushing Syndrome, Female, Forecasting, Humans, Male, Middle Aged, Cohort Studies, Survival Analysis, Age of Onset, Kaplan-Meier Estimate, Young Adult
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