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

Ragnarsson, Oskar ; Olsson, Daniel s ; Papakokkinou, Eleni ; Chantzichristos, Dimitrios ; Dahlqvist, Per ; Segerstedt, Elin ; Olsson, Tommy ; Petersson, Maria ; Berinder, Katarina and Bensing, Sophie , et al. (2019) In The Journal of clinical endocrinology and metabolism 104(6). p.2375-2384
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... (More)
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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publishing date
type
Contribution to journal
publication status
published
subject
in
The Journal of clinical endocrinology and metabolism
volume
104
issue
6
pages
2375 - 2384
publisher
Oxford University Press
external identifiers
  • scopus:85064489296
ISSN
1945-7197
DOI
10.1210/jc.2018-02524
language
English
LU publication?
no
id
45845e17-8f98-4374-8997-a0d79f789f51
date added to LUP
2023-11-13 11:36:06
date last changed
2024-02-26 11:44:33
@article{45845e17-8f98-4374-8997-a0d79f789f51,
  abstract     = {{Context: Whether patients with Cushing disease (CD) in remission have increased mortality is still debatable.<br/>Objective: To study overall and disease-specific mortality and predictive factors in an unselected nationwide cohort of patients with CD.<br/>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.<br/>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.<br/><br/>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.<br/>}},
  author       = {{Ragnarsson, Oskar and Olsson, Daniel s and Papakokkinou, Eleni and Chantzichristos, Dimitrios and Dahlqvist, Per and Segerstedt, Elin and Olsson, Tommy and Petersson, Maria and Berinder, Katarina and Bensing, Sophie and Höybye, Charlotte and Edén-Engström, Britt and Burman, Pia and Bonelli, Lorenza and Follin, Cecilia and Petranek, David and Erfurth, Eva Marie and Wahlberg, Jeanette and Ekman, Bertil and Åkerman, Anna-Karin and Schwarcz, Erik and Bryngelsson, Ing-Liss and Johannsson, Gudmundur}},
  issn         = {{1945-7197}},
  language     = {{eng}},
  month        = {{06}},
  number       = {{6}},
  pages        = {{2375--2384}},
  publisher    = {{Oxford University Press}},
  series       = {{The Journal of clinical endocrinology and metabolism}},
  title        = {{Overall and Disease-Specific Mortality in Patients With Cushing Disease: A Swedish Nationwide Study}},
  url          = {{http://dx.doi.org/10.1210/jc.2018-02524}},
  doi          = {{10.1210/jc.2018-02524}},
  volume       = {{104}},
  year         = {{2019}},
}