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. 2019 Apr;22(2):179-186.
doi: 10.1007/s11102-019-00951-1.

The incidence of Cushing's disease: a nationwide Swedish study

Oskar Ragnarsson  1 , Daniel S Olsson  2 , Dimitrios Chantzichristos  2 , Eleni Papakokkinou  2 , Per Dahlqvist  3 , Elin Segerstedt  3 , Tommy Olsson  3 , Maria Petersson  4   5 , Katarina Berinder  4   5 , Sophie Bensing  4   5 , Charlotte Höybye  4   5 , Britt Edén Engström  6 , Pia Burman  7   8 , Lorenza Bonelli  7   8 , Cecilia Follin  9 , David Petranek  9 , Eva Marie Erfurth  9 , Jeanette Wahlberg  10 , Bertil Ekman  10 , Anna-Karin Åkerman  11 , Erik Schwarcz  11 , Ing-Liss Bryngelsson  12 , Gudmundur Johannsson  2
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The incidence of Cushing's disease: a nationwide Swedish study

Oskar Ragnarsson et al. Pituitary. 2019 Apr.

Abstract

Background: Studies on the incidence of Cushing's disease (CD) are few and usually limited by a small number of patients. The aim of this study was to assess the annual incidence in a nationwide cohort of patients with presumed CD in Sweden.

Methods: Patients registered with a diagnostic code for Cushing's syndrome (CS) or CD, between 1987 and 2013 were identified in the Swedish National Patient Registry. The CD diagnosis was validated by reviewing clinical, biochemical, imaging, and histopathological data.

Results: Of 1317 patients identified, 534 (41%) had confirmed CD. One-hundred-and-fifty-six (12%) patients had other forms of CS, 41 (3%) had probable but unconfirmed CD, and 334 (25%) had diagnoses unrelated to CS. The mean (95% confidence interval) annual incidence between 1987 and 2013 of confirmed CD was 1.6 (1.4-1.8) cases per million. 1987-1995, 1996-2004, and 2005-2013, the mean annual incidence was 1.5 (1.1-1.8), 1.4 (1.0-1.7) and 2.0 (1.7-2.3) cases per million, respectively. During the last time period the incidence was higher than during the first and second time periods (P < 0.05).

Conclusion: The incidence of CD in Sweden (1.6 cases per million) is in agreement with most previous reports. A higher incidence between 2005 and 2013 compared to 1987-2004 was noticed. Whether this reflects a truly increased incidence of the disease, or simply an increased awareness, earlier recognition, and earlier diagnosis can, however, not be answered. This study also illustrates the importance of validation of the diagnosis of CD in epidemiological research.

Keywords: Cushing’s syndrome; Epidemiology; Incidence; Validation.

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Figures

Fig. 1
Fig. 1
Age distribution at first diagnosis in patients with confirmed CD in Sweden
Fig. 2
Fig. 2
Annual incidence (cases per 1 million inhabitants per year) of CD in Sweden between 1987 and 2013
See this image and copyright information in PMC

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