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Overall and disease-specific mortality in patients with Cushing's disease: a Swedish nationwide study.

Artikel i vetenskaplig tidskrift
Författare Oskar Ragnarsson
Daniel S Olsson
Eleni Papakokkinou
Dimitrios Chantzichristos
Per Dahlqvist
Elin Segerstedt
Tommy Olsson
Maria Petersson
Katarina Berinder
Sophie Bensing
Charlotte Höybye
Britt Edén-Engström
Pia Burman
Lorenza Bonelli
Cecilia Follin
David Petranek
Eva Marie Erfurth
Jeanette Wahlberg
Bertil Ekman
Anna-Karin Åkerman
Erik Schwarcz
Ing-Liss Bryngelsson
Gudmundur Johannsson
Publicerad i The Journal of clinical endocrinology and metabolism
Volym 104
Nummer/häfte 6
Sidor 2375–2384
ISSN 1945-7197
Publiceringsår 2019
Publicerad vid Institutionen för medicin, avdelningen för invärtesmedicin och klinisk nutrition
Sidor 2375–2384
Språk en
Länkar dx.doi.org/10.1210/jc.2018-02524
www.ncbi.nlm.nih.gov/entrez/query.f...
Ämneskategorier Endokrinologi

Sammanfattning

It is still a matter of debate whether patients with Cushing's disease (CD) in remission have increased mortality.To study overall and disease-specific mortality, and predictive factors, in an unselected nationwide cohort of patients with CD.A retrospective study on patients diagnosed with CD, identified in the Swedish National Patient Registry between 1987 and 2013. Medical records were systematically reviewed to verify the diagnosis. Standardised mortality ratios (SMRs) with 95% confidence intervals (CI) were calculated and Cox regression models were used to identify predictors of mortality.Five-hundred-and-two patients [387 women (77%)] with CD were identified, of whom 419 (83%) were confirmed to be in remission. Mean age at diagnosis was 43 years (SD 16) and median follow-up time was 13 years (IQR 6-23). The observed number of deaths was 133 versus 54 expected, resulting in an overall SMR of 2.5 (95% CI 2.1-2.9). The commonest cause of death was cardiovascular diseases [SMR 3.3 (95% CI 2.6 -4.3)]. Excess mortality was also found due to infections and suicides. SMR in patients in remission was 1.9 (95% CI 1.5-2.3), where bilateral adrenalectomy and glucocorticoid replacement therapy were independently associated with increased mortality whereas growth hormone replacement was associated with improved outcome.This large nationwide study shows that patients with CD have an excess mortality. The findings illustrate the importance of obtaining remission and continued active surveillance, along with adequate hormone replacement, and evaluation of cardiovascular risk and mental health.

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