Síndrome de cushing endógena: características clínico-laboratoriais em 73 casos

Abstract

We studied clinical and laboratorial features of 73 patients with endogenous Cushing's syndrome, subdivided as follows: 46 (63%) with Cushing's disease (CD), 21 (28.7%) with an adrenal tumor and 6 (8.2%) with ectopic ACTH secretion (EAS). The rate of typical manifestations of hypercortisolism was similar regardless its etiology. In 100% of cases of Cushing's syndrome we observed serum cortisol levels greater than 1.8 microg/dL in low-dose dexamethasone (DMS) suppression tests, as well as elevation of serum or salivary midnight cortisol. However, urinary free cortisol was normal in 11.5% of patients. ACTH levels were suppressed in patients with adrenal tumors, normal or high in CD and always high in EAS. In the 8-mg overnight DMS suppression test, serum cortisol suppression > 50% was observed in 78.2% of cases of CD and in 33.3% of subjects with EAS, while an 80% suppression was only seen in CD. After stimulation with CRH or DDAVP an ACTH increase > 35% occurred in 81% of individuals with CD and 16.6% of those with EAS, while an ACTH increase > 50 achieved 100% specificity. Moreover, the combination of serum cortisol suppression > 50% and an ACTH increase > 35% in both tests only occurred in Cushing's disease. Pituitary magnetic resonance imaging identified 100% of macroadenomas and 59.4% of microadenomas in patients with CD. Among 10 patients that underwent bilateral inferior petrosal sinus sampling, a central-to-peripheral ACTH gradient > 3 after CRH or DDAVP had 90% sensitivity and 100% specificity for Cushing's disease.

Síndrome De Cushing Iatrogênica

“…8 A Doença de Cushing (DC) é o nome dado ao hipercortisolismo endógeno causado pela elevada secreção de hormônio adrenocorticotrófico (ACTH) por lesão hipofisária. 9 O tumor produtor de ACTH presente na DC não se sensibiliza aos feedbacks que ocorreriam no funcionamento normal, o que torna o sistema não mais autorregulável, e em consequência disso, os níveis de glicocorticoides ficam aumentados, gerando o hipercortisolismo. 1,11 Síndrome de Cushing Iatrogênica (SCI) ocorre por uma desordem provocada pelo uso excessivo ou prolongado de corticosteroides.…”

Síndrome de cushing e hipercortisolismo associado: uma revisão literária / Cushing’s syndrome and hypercortisolism associated: review of literature

“…Esse diagnóstico envolve etapas da confirmação do hipercortisolismo e investigação de sua causa. Muitas vezes há omissão de informações do paciente sobre o uso de preparações tópicas, colírios e gotas nasais de glicocorticoides, o qual é um ponto chave de investigação para exclusão da SC 8 . Síndrome de Cushing foi descrita pela primeira vez em 1932 por um neurocirurgião norte americano que homenageou a doença com o seu sobrenome.…”

Long Term Follow-up after Endoscopic Endonasal Approach for the Treatment of Cushing's Disease

“…It is more frequently diagnosed during the fourth decade of life, in slightly younger females (mean 30.5 years) than males (mean 37.1 years), with a preference for the female gender (3-5:1). [1][2][3][4][5] The most common clinical features are obesity, diabetes mellitus, hypertension, and facial plethora. Psychiatric changes (depression, emotional lability, anxiety, psychosis, panic attacks, suicidal ideation, and paranoia) and neurocognitive deficits (learning impairment and memory deficits) can also be observed.…”

“…Psychiatric changes (depression, emotional lability, anxiety, psychosis, panic attacks, suicidal ideation, and paranoia) and neurocognitive deficits (learning impairment and memory deficits) can also be observed. 2,3,5 If untreated, Cushing's disease is associated a high morbidity rate from cardiac and cerebrovascular events, immunosuppression, osteoporosis, and psychiatric disturbances, leading to a mortality rate 1.9-4.8 times higher than the general population. 2,3 Cushing's disease is caused by a benign monoclonal pituitary corticotroph adenoma that secretes excessive adrenocorticotropic hormone (ACTH), leading to continuous supraphysiological secretion of glucocorticoids from the adrenal glands.…”

“…2,3,5 If untreated, Cushing's disease is associated a high morbidity rate from cardiac and cerebrovascular events, immunosuppression, osteoporosis, and psychiatric disturbances, leading to a mortality rate 1.9-4.8 times higher than the general population. 2,3 Cushing's disease is caused by a benign monoclonal pituitary corticotroph adenoma that secretes excessive adrenocorticotropic hormone (ACTH), leading to continuous supraphysiological secretion of glucocorticoids from the adrenal glands. Therefore, the circadian variation is lost and there is a negative feedback inhibition on corticotropin releasing hormone (CRH) secretion by the hypothalamus.…”