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Overall and Disease-Specific Mortality in Patients With Cushing Disease: A Swedish Nationwide Study
Oskar Ragnarsson1,2, Daniel S Olsson1,2, Eleni Papakokkinou1,2
1Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, Göteborg, Sweden.
Insights
Patients with Cushing disease (CD) have higher mortality rates, even in remission. Cardiovascular diseases, infections, and suicide are leading causes, highlighting the need for ongoing monitoring and management.
Area of Science:
- Endocrinology
- Epidemiology
- Clinical Medicine
Background:
- Cushing disease (CD) is a rare endocrine disorder caused by excess cortisol production.
- The long-term mortality risk for patients with CD, particularly those in remission, remains incompletely understood.
Purpose of the Study:
- To investigate overall and disease-specific mortality in a nationwide cohort of patients with Cushing disease.
- To identify predictors of mortality in patients with CD.
Main Methods:
- Retrospective analysis of patients diagnosed with CD between 1987 and 2013 in the Swedish National Patient Registry.
- Systematic medical record review for diagnosis verification.
- Calculation of standardized mortality ratios (SMRs) and Cox regression modeling.
Main Results:
- The study included 502 patients with CD, 83% of whom achieved remission.
- Overall mortality was significantly increased (SMR 2.5), with cardiovascular diseases being the most common cause of death.
- In remission, mortality remained elevated (SMR 1.9), with bilateral adrenalectomy and glucocorticoid replacement linked to increased risk, while GH replacement showed a protective effect.
Conclusions:
- Patients with Cushing disease experience excess mortality, underscoring the importance of achieving and maintaining remission.
- Continued surveillance, adequate hormone replacement, and management of cardiovascular and mental health are crucial for improving outcomes in CD patients.
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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