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

Ragnarsson, Oskar; Olsson, Daniel S.; Chantzichristos, Dimitrios; Papakokkinou, Eleni; Dahlqvist, Per; Segerstedt, Elin; Olsson, Tommy; Petersson, Maria; Berinder, Katarina and Bensing, Sophie, et al. (2019) In Pituitary
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%... (More)

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.

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@article{591539c6-7d9e-4c4c-a3ec-174d8c4268b0,
  abstract     = {<p>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 &lt; 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.</p>},
  author       = {Ragnarsson, Oskar and Olsson, Daniel S. and Chantzichristos, Dimitrios and Papakokkinou, Eleni 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         = {1386-341X},
  keyword      = {Cushing’s syndrome,Epidemiology,Incidence,Validation},
  language     = {eng},
  month        = {02},
  publisher    = {Springer},
  series       = {Pituitary},
  title        = {The incidence of Cushing’s disease : a nationwide Swedish study},
  url          = {http://dx.doi.org/10.1007/s11102-019-00951-1},
  year         = {2019},
}