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Endocrine Abstracts (2017) 49 GP9 | DOI: 10.1530/endoabs.49.GP9

1Institute of Medicine at Sahlgrenska Academy, University of Gothenburg and the Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden; 2Department of Medicine, Umeå University, Umeå, Sweden; 3Department of Molecular Medicine and Surgery, Karolinska Institutet, and Department of Endocrinology, Metabolism and Diabetology, Karolinska University Hospital, Stockholm, Sweden; 4Department of Medical Sciences; Endocrinology, Diabetes and Metabolism, Uppsala University Hospital, Uppsala, Sweden; 5Department of Endocrinology, Skane University Hospital, Malmö, University of Lund, Malmö, Sweden; 6Department of Endocrinology, Skane University Hospital, Lund, Sweden; 7Department of Endocrinology and Department of Medical and Health Sciences, Linköping University, Linköping, Sweden; 8Department of Internal Medicine, School of Health and Medical Sciences, Örebro University, Örebro, Sweden; 9Department of Occupational and Environmental Medicine, Örebro University Hospital, Örebro, Sweden; 10Department of Occupational and Environmental Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg and Sahlgrenska University Hospital, Gothenburg, Sweden.


Background: It is still undetermined whether patients with Cushing’s disease (CD) in remission have an increased mortality. Most previous studies are limited by small numbers of patients and/or short follow-up time.

Objective: To study mortality in a nation-wide cohort of patients with CD during long-term follow-up.

Methods: Patients with ICD codes for Cushing’s syndrome and/or CD, between 1987 and 2013, were identified in the Swedish National Patient Registry. Medical records of the patients were reviewed (clinical, biochemical, imaging and histopathological data) to verify the diagnosis, and to determine remission status. All other forms of Cushing’s syndrome than CD were excluded from the study. Standardized mortality ratios (SMRs) with 95% confidence intervals were calculated using the Swedish general population as reference.

Results: Out of 1252 identified patients, 502 (387 women (77%)) had a confirmed CD diagnosis. Of these, 411 (82%) were in biochemical remission. The mean±S.D. age at diagnosis was 46±16 years. The median (interquartile range) follow-up time was 13 (6–23) years, resulting in 7165 patient-years of follow-up. Of the 502 patients, 364 (73%) had been treated with pituitary surgery, 129 (26%) with radiotherapy and 102 (20%) with bilateral adrenalectomy. The observed number of deaths was 133 vs 54 expected, resulting in an overall SMR of 2.46 (95% CI 2.06–2.91). SMR in patients not in remission (n=91) was markedly increased, SMR 8.23 (95% CI 5.42–12.0). Mortality in patients in biochemical remission was lower (P<0.0001), but increased compared to the general population, SMR 1.80 (95% CI 1.44–2.23).

Conclusion: This large nation-wide study demonstrates that patients with CD have an excess mortality, irrespective of remission status. The findings emphasize the importance of curative treatment as well as continued active surveillance after remission has been achieved.

Volume 49

19th European Congress of Endocrinology

Lisbon, Portugal
20 May 2017 - 23 May 2017

European Society of Endocrinology 

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