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

Research output: Contribution to journalArticle


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.


  • Oskar Ragnarsson
  • Daniel S. Olsson
  • Dimitrios Chantzichristos
  • Eleni Papakokkinou
  • Per Dahlqvist
  • Elin Segerstedt
  • Tommy Olsson
  • Maria Petersson
  • Katarina Berinder
  • Sophie Bensing
  • Charlotte Höybye
  • Britt Edén Engström
  • Lorenza Bonelli
  • David Petranek
  • Jeanette Wahlberg
  • Bertil Ekman
  • Anna Karin Åkerman
  • Erik Schwarcz
  • Ing Liss Bryngelsson
  • Gudmundur Johannsson
External organisations
  • Sahlgrenska University Hospital
  • Umeå University
  • Karolinska University Hospital
  • Karolinska Institutet
  • Uppsala University Hospital
  • Skåne University Hospital
  • Linköping University
  • Örebro University
  • Örebro University Hospital
  • University of Gothenburg
  • Lund University
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Endocrinology and Diabetes


  • Cushing’s syndrome, Epidemiology, Incidence, Validation
Original languageEnglish
Pages (from-to)179-186
Issue number2
Early online date2019 Feb 25
Publication statusPublished - 2019
Publication categoryResearch