Cushing’s disease epidemiology and demographics

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Cushing's disease (also known as Cushing disease, tertiary or secondary hypercortisolism, tertiary or secondary hypercorticism, Itsenko-Cushing disease)[1][2] is a cause of Cushing's syndrome characterised by increased secretion of adrenocorticotropic hormone (ACTH) from the anterior pituitary (secondary hypercortisolism). This is most often as a result of a pituitary adenoma (specifically pituitary basophilism) or due to excess production of hypothalamus CRH (Corticotropin releasing hormone) (tertiary hypercortisolism/hypercorticism) that stimulates the synthesis of cortisol by the adrenal glands. Pituitary adenomas are responsible for 80% of endogenous Cushing's syndrome,[3] when excluding Cushing's syndrome from exogenously administered corticosteroids. This should not be confused with ectopic Cushing syndrome or exogenous steroid use.[4]


Cases of Cushing's disease are rare, and few epidemiological data is available on the disease. An 18 year study conducted on the population of Vizcaya, Spain reported a 0.004% prevalence of Cushing's disease.[5] The average incidence of newly diagnosed cases was 2.4 cases per million inhabitants per year. The disease is often diagnosed 3–6 years after the onset of illness.[5] Several studies have shown that Cushing's disease is more prevalent in women than men at a ratio of 3-6:1, respectively.[6][7] Moreover, most women affected were between the ages of 50 and 60 years. The prevalence of hypertension, and abnormalities in glucose metabolism are major predictors of mortality and morbidity in untreated cases of the disease.[5] The mortality rate of Cushing's disease was reported to be 10-11%,[5][8] with the majority of deaths due to vascular disease [9][5] Women aged 45–70 years have a significantly higher mortality rate than men.[5] Moreover, the disease shows a progressive increase with time. Reasons for the trend are unknown, but better diagnostic tools, and a higher incidence rate are two possible explanations.[5]



  1. "Whonamedit - Nikolai Mikhailovich Itsenko". "Nikolai Mikhailovich Itsenko investigated neural infections, vegetative nervous system diseases and cerebral tumors. In 1926 he was the first one who described Itsenko-Cushing's disease, six years before Cushing."
  2. A.I. Gozhenko, I.P. Gurkalova, W. Zukow, Z. Kwasnik, B. Mroczkowska (2009). "Trematoda". Pathology: Medical Student's Library. Radomska Szkola Wyžsza uk. Zubrzyckiego. p. 280. ISBN 978-83-61047-18-6.
  3. Lanzino, Giuseppe; Maartens, Niki F.; Laws, Edward R. (2002). "Cushing's case XLV: Minnie G.". Journal of Neurosurgery. 97 (1): 231–234. doi:10.3171/jns.2002.97.1.0231. PMID 12134925. |access-date= requires |url= (help)
  5. 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Etxabe, J.; J. A. Vazquez (1994). "Morbidity and mortality in Cushing's disease: an epidemiological approach". Clinical endocrinology. 40 (4): 479–484. doi:10.1111/j.1365-2265.1994.tb02486.x. PMID 8187313. |access-date= requires |url= (help)
  6. Boggan, J.E; Tyrell, J.B; Wilson C.B (1983). "Transsphenoidal microsurgical management of Cushing's disease: report of 100 cases". Journal of neurosurgery. 95 (2): 195–200. doi:10.3171/jns.1983.59.2.0195.
  7. Howlet, T.A; Perry L.; Doniach I.; Rees LH.; Besser G.M (1986). "Diagnosis and management of ACTHdependent Cushing's syndrome: comparison of the features in ectopic and pituitary ACTH production". Clinical endocrinology. 24 (6): 699–713. doi:10.1111/j.1365-2265.1986.tb01667.x. PMID 3024870. Retrieved January 31, 2014.
  8. Lindholm, J.; Juul, S.; Jørgensen, J.O.L.; Astrup, J.; Bjerre, P.; Feldt-Rasmussen, U.; Hagen, C.; Jørgensen, J.; Kosteljanetz, M.; Kristensen, L.Ø.; Laurberg, P.; Schmidt, K.; Weeke, J (2001). "Incidence and late prognosis of Cushing's syndrome: A population-based study". Journal of Clinical Endocrinology and Metabolism. 86 (1): 117–123. doi:10.1210/jc.86.1.117. PMID 11231987. Retrieved January 31, 2014.
  9. 9.0 9.1 Wilson, P.J.; Williams, J.R.; Smee, R.I. (2014). "Cushing's disease: A single centre's experience using the linear accelerator (LINAC) for stereotactic radiosurgery and fractionated stereotactic radiotherapy". Journal of Clinical Neuroscience. 21 (1): 100–106. doi:10.1016/j.jocn.2013.04.007. PMID 24074805.