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

Journal article
Authors 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
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
Published in Pituitary
Volume 22
Issue 2
Pages 179–186
ISSN 1573-7403
Publication year 2019
Published at Institute of Medicine
Institute of Medicine, Department of Internal Medicine and Clinical Nutrition
Pages 179–186
Language en
Links dx.doi.org/10.1007/s11102-019-00951...
www.ncbi.nlm.nih.gov/entrez/query.f...
Subject categories Endocrinology

Abstract

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.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.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).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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