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

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

Author information

1
Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, and The Department of Endocrinology, Sahlgrenska University Hospital, Gröna Stråket 8, 413 45, Gothenburg, Sweden. oskar.ragnarsson@medic.gu.se.
2
Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, and The Department of Endocrinology, Sahlgrenska University Hospital, Gröna Stråket 8, 413 45, Gothenburg, Sweden.
3
Department of Medicine, Umeå University, 901 87, Umeå, Sweden.
4
Patient Area Endocrinology and Nephrology, Inflammation and Infection Theme, Karolinska University Hospital, 171 76, Solna, Sweden.
5
Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 77, Stockholm, Sweden.
6
Department of Medical Sciences, Endocrinology, Diabetes and Metabolism, Uppsala University Hospital, 751 85, Uppsala, Sweden.
7
Department of Endocrinology, Skåne University Hospital, 214 28, Malmö, Sweden.
8
University of Lund, 223 50, Lund, Sweden.
9
Department of Endocrinology, Skåne University Hospital, 222 42, Lund, Sweden.
10
Department of Endocrinology, Department of Medical and Health Sciences, Linköping University, 581 83, Linköping, Sweden.
11
Department of Internal Medicine, School of Medical Sciences, Örebro University, 702 81, Örebro, Sweden.
12
Department of Occupational and Environmental Medicine, Örebro University Hospital, 702 81, Örebro, Sweden.

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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