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; Bensing, Sophie; Höybye, Charlotte; Engström, Britt Edén; Burman, Pia; Bonelli, Lorenza; Follin, Cecilia; Petranek, David; Erfurth, Eva Marie; Wahlberg, Jeanette; Ekman, Bertil; Åkerman, Anna Karin; Schwarcz, Erik; Bryngelsson, Ing Liss; Johannsson, Gudmundur

doi:10.1007/s11102-019-00951-1

Cited by 60 publications

(42 citation statements)

References 25 publications

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“…The incidence of CD in our study (1.5 cases per million/y) is in line most previous studies . Similarly, the incidence of cortisol‐producing adrenal adenoma (0.5 cases per million/y) is close to the incidence reported in the two studies that have included patients with various forms of CS, not only CD (0.3 and 0.6 cases per million/y, respectively; Table ) .…”

Section: Discussionsupporting

confidence: 86%

“…The incidence of CD in our study (1.5 cases per million/y) is in line most previous studies. [2][3][4][5][6][7][8][9] Similarly, the incidence of cortisol-producing adrenal adenoma (0.5 cases per million/y) is close to the incidence reported in the two studies that have included patients with various forms of CS, not only CD (0.3 and 0.6 cases per million/y, respectively; Table 2). 8,9 On the contrary, we found a considerably higher incidence of ectopic CS (0.8 cases per million/y) than the two previously mentioned studies (0.1 and 0.3 cases per million/y, respectively).…”

Section: Discussionsupporting

confidence: 72%

“…Cushing's syndrome (CS) is a rare endocrine disorder caused by a prolonged exposure to elevated cortisol levels . Due to its rarity, and heterogeneity, epidemiological studies on the incidence of CS are few and most often only includes patients with the most common form, that is Cushing's disease (CD) . In fact, only two population‐based studies have evaluated the incidence of other aetiologies .…”

Section: Introductionmentioning

confidence: 99%

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The incidence of endogenous Cushing's syndrome in the modern era

Wengander

Trimpou

Papakokkinou

et al. 2019

Clinical Endocrinology

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Introduction Epidemiological studies on the incidence of Cushing's syndrome (CS) are few and most often includes only patients with the most common form, that is Cushing's disease (CD). Objective To analyse the incidence of endogenous CS in an unselected cohort of patients from western Sweden between 2002 and 2017. Methods Medical records from patients who had received a diagnostic code for CS and adrenocortical carcinoma in the Västra Götaland County between 2002 and 2017 were reviewed. Eighty‐two patients had been diagnosed with endogenous CS in the region during the study period and were included in this analysis. Results Thirty‐nine patients (48%) had CD, 21 (26%) had ectopic ACTH‐producing tumour, 17 (21%) had a benign adrenal CS, and 5 (6%) had cortisol‐producing adrenocortical carcinoma. Nine of 21 (43%) patients with ectopic CS had an ACTH‐producing lung tumour, 4 had a neuroendocrine pancreas tumour, and 5 had an occult tumour. The annual incidence of endogenous CS was 3.2 cases per million/y; 1.5 for CD, 0.8 for ectopic CS, 0.7 for benign adrenal CS and 0.2 for adrenocortical carcinoma. Conclusions Approximately three new cases of endogenous CS, per one million inhabitants, were diagnosed annually between 2002 and 2017. Compared with previous reports, proportionally more patients had ectopic CS. The reason for this is unclear although increased awareness of hypercortisolism in patients with malignant tumours in the modern era is a possible explanation.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionsupporting

confidence: 72%

Section: Introductionmentioning

confidence: 99%

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The incidence of endogenous Cushing's syndrome in the modern era

Wengander

Trimpou

Papakokkinou

et al. 2019

Clinical Endocrinology

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“…Cushing's disease (CD) is a rare condition of cortisol excess attributed to a pituitary adenoma. Epidemiological studies suggest an annual incidence of 1.2-2.4 cases per million population [1][2][3][4][5][6][7] and a prevalence of 12-79 cases per 1 000 000 [1,4,6,[8][9][10]. Median age at diagnosis is 40 years (range 10-76) [11,12] with a clear female preponderance (73-93 % of the patients in published series are women) [4,11,[13][14][15][16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 95%

Mortality of Patients with Cushing’s Disease

Ntali

Hakami

Wattegama

et al. 2020

Exp Clin Endocrinol Diabetes

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AbstractCushing’s disease is a rare condition of cortisol excess attributed to a pituitary adenoma with an annual incidence of 1.2–2.4 cases per million population. It is associated with several co-morbidities leading to increased mortality predominantly due to cardiovascular disease. Despite the advances in its diagnosis and management, survival can be compromised even after apparent successful treatment. Minimizing the duration and extent of exposure to hypercortisolaemia by early diagnosis and rapid, effective therapeutic interventions, as well as close monitoring and aggressive control of cardiovascular risk factors are vital for improving outcomes of the patients.

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“…Therefore, during a COVID-19 pandemic, it could be argued that CS patients may be at high risk of developing COVID-19 infection, as well as potentially experiencing a more severe clinical course due to their chronic metabolic comorbidities, which usually improve but are often not fully normalized after hypercortisolism resolution [6]. However, since CS has an estimated prevalence of around 40 cases per million and an estimated incidence of 0.7-2.4 cases per million per year, although the worldwide epidemiology has not been fully determined [8], up to now, there have been no reports of COVID-19 infections in CS patients.…”

Section: Introductionmentioning

confidence: 99%

Glucocorticoid excess and COVID-19 disease

Guarnotta

Ferrigno

Martino

et al. 2020

Rev Endocr Metab Disord

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The pandemic of coronavirus disease (COVID-19), a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is causing high and rapid morbidity and mortality. Immune system response plays a crucial role in controlling and resolving the viral infection. Exogenous or endogenous glucocorticoid excess is characterized by increased susceptibility to infections, due to impairment of the innate and adaptive immune system. In addition, diabetes, hypertension, obesity and thromboembolism are conditions overrepresented in patients with hypercortisolism. Thus patients with chronic glucocorticoid (GC) excess may be at high risk of developing COVID-19 infection with a severe clinical course. Care and control of all comorbidities should be one of the primary goals in patients with hypercortisolism requiring immediate and aggressive treatment. The European Society of Endocrinology (ESE), has recently commissioned an urgent clinical guidance document on management of Cushing’s syndrome in a COVID-19 period. In this review, we aim to discuss and expand some clinical points related to GC excess that may have an impact on COVID-19 infection, in terms of both contagion risk and clinical outcome. This document is addressed to all specialists who approach patients with endogenous or exogenous GC excess and COVID-19 infection.

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

Cited by 60 publications

References 25 publications

The incidence of endogenous Cushing's syndrome in the modern era

The incidence of endogenous Cushing's syndrome in the modern era

Mortality of Patients with Cushing’s Disease

Glucocorticoid excess and COVID-19 disease

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