[Prevalence, etiology and clinical findings of Cushing's syndrome].
Endogenous Cushing's syndrome is a very rare entity, with an incidence of 2-4 cases per million inhabitants per year. Cases caused by ectopic ACTH secretion are under-diagnosed. Cushing's disease is the most frequent cause of endogenous Cushing's syndrome, which is 5 or 6 times more frequent than adrenal Cushing's syndrome, with an incidence of between 1.2 and 2.4 cases per million inhabitants per year. Cushing's disease is 3-8 times higher in women than in men. The frequency of adrenal tumors is 3 times higher in women, while that of Cushing's syndrome due to adrenal tumors is 3-5 times higher. Age at diagnosis of Cushing's syndrome varies according to the etiology. Most cases of Cushing's disease are due to a pituitary adenoma, although the tumor may not be visible on the available imaging techniques. ACTH-independent Cushing's syndrome is found in 20% of cases and is most frequently due to adenomas (10%) or adrenal carcinomas (8). Bilateral micronodular hyperplasia and macronodular hyperplasia are infrequent entities, representing less than 10% of all cases of ACTH-independent Cushing's syndrome. Both familial and sporadic forms exist: the familial form, or Carney complex, and ACTH-independent bilateral macronodular hyperplasia, in which the size of the adrenal glands is considerably enlarged. The signs and symptoms of Cushing's syndrome are a direct result of long-term exposure to excessive glucocorticoids. Most signs and symptoms are highly prevalent in the general population (hypertension, central obesity, diabetes mellitus or carbohydrate intolerance, osteoporosis, and characteristic phenotypical alterations).
Lahera Vargas
Marcos
M
Servicio de Endocrinología, Hospital Ramón y Cajal, Madrid, España. marcos.lahera@salud.madrid.org
da Costa
César Varela
CV
D000182:ACTH Syndrome, Ectopic / Q000150:complications; D000236:Adenoma / Q000150:complications / Q000378:metabolism; D000302:Adrenal Cortex / Q000473:pathology; D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000378:metabolism; D002277:Carcinoma / Q000150:complications / Q000378:metabolism; D002318:Cardiovascular Diseases / Q000453:epidemiology; D003480:Cushing Syndrome / Q000453:epidemiology / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D003920:Diabetes Mellitus / Q000453:epidemiology; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D006965:Hyperplasia; D015994:Incidence; D008297:Male; D010641:Phenotype; D047748:Pituitary ACTH Hypersecretion / Q000150:complications; D010911:Pituitary Neoplasms / Q000150:complications / Q000378:metabolism; D015995:Prevalence; D012306:Risk
D005938:Glucocorticoids; D006854:Hydrocortisone