Recent Updates on the Diagnosis and Management of Cushing's Syndrome

Abstract

Cushing's syndrome, a potentially lethal disorder characterized by endogenous hypercortisolism, may be difficult to recognize, especially when it is mild and the presenting features are common in the general population. However, there is a need to identify the condition at an early stage, as it tends to progress, accruing additional morbidity and increasing mortality rates. Once a clinical suspicion is raised, screening tests involve timed measurement of urine, serum or salivary cortisol at baseline or after administration of dexamethasone, 1 mg. Each test has caveats, so that the choice of tests must be individualized for each patient. Once the diagnosis is established, and the cause is determined, surgical resection of abnormal tumor/tissue is the optimal treatment. When this cannot be achieved, medical treatment (or bilateral adrenalectomy) must be used to normalize cortisol production. Recent updates in screening for and treating Cushing's syndrome are reviewed here.

Prospective Evaluation of Late-Night Salivary Cortisol and Cortisone by EIA and LC-MS/MS in Suspected Cushing Syndrome

“…Since sensitivity should be maximized in screening tests, the excellent sensitivity and negative predictive value of LNSC makes it a valuable screening tool. However, other clinical findings and biochemical tests, including DST, are needed to provide confirmation of CD [ 18 , 24 , 45 , 87 ].…”

Glucocorticoid Receptor Antagonist Administration Prevents Adrenal Gland Atrophy in an ACTH-Independent Cushing’s Syndrome Rat Model

“…The selection of drugs for the preoperative treatment of ACTH-independent CS was considered from the viewpoint of the preservation of adrenal function after surgery as follows. Currently, there are several medications for CS, and adrenal steroidogenesis inhibitors such as mitotane and metyrapone are used to treat this disease [21]. Mitotane is a dichlorodiphenyltrichloroethane (DDT) derivative that acts on the adrenal mitochondria, exhibiting adrenocortical injury, and causing irreversible damage to the adrenal cortex [22].…”

Cirurgia poupadora de adrenal: uma nova abordagem no tratamento da hiperplasia macronodular adrenal primária

“…Twelve patients (70.5%) recovered adrenal function in cortisol production, while the other 5 remained in adrenal insufficiency The average time to adrenal function recovery was 32 months. In post-operative CT the average adrenal volume was 4.1 cm 3 . Measurable ACTH level (not suppressed) was associated with a better chance to recover hormonal function (p=0.003).…”

“…Com o objetivo de evitar os inconvenientes da reposição hormonal permanente e, ao mesmo tempo, minimizar os risco de hipercortisolismo, estudamos uma série de pacientes com HMAP tratados com adrenalectomia laparoscópica da maior glândula (ou com maior funcionalidade) simultânea à adrenalectomia parcial laparoscópica contralateral, procedimento que denominamos de cirurgia poupadora de adrenal. Onze pacientes (65%) apresentavam supressão da produção de ACTH e todos os 17 pacientes da amostra apresentaram ausência de supressão da produção de cortisol após o teste de supressão com 1mg de dexametasona (cortisol >1,8μg/L), com valor médio do cortisol sérico de 14,5μg/dL (3,8). Apenas um dos pacientes da casuística não fazia uso de medicação para hipertensão arterial, 11 deles faziam tratamento para diabete melito (65%) e nove para dislipidemia (53%).…”

“…O tempo médio de recuperação da função da adrenal foi de 32 meses. O volume médio de glândula adrenal remanescente foi de 4,1 cm 3 . O nível sérico de ACTH mensurável se mostrou um fator preditivo de menor tempo de recuperação da função hormonal (p=0,003).…”