Hiperplasia suprarrenal macronodular bilateral independiente de ACTH: caso clínico / ACTH independent bilateral macronodular adrenal hyperplasia: report of one case

ABSTRACT

ACTH Independent cushing syndrome, involving both adrenal glands, represents a minority of endogenous hyperadrenocorticisms and is caused by infrequent diseases. ACTH independent macronodular adrenal hyperplasia (AIMAH) is one of them and is caused by aberrant cortical adrenal receptors expression that are able to stimulate cortisol secretion. We report a 62 years old male, presenting with hypertension, weight gain, urinary lithiasis and osteopenia. The diagnosis of Cushing's syndrome was made with a morning cortisol level after 1 mg nocturnal dexamethasone of 8.2 ug/dL. Urinary free 24 hour cortisol levels were normal, circadian rhythm of cortisol was maintained and ACTH was <5 pg/mL. Abdominal magnetic resonance imaging showed bilateral nodular adrenal enlargement. The functional study, looking for abnormal receptors, showed a potent cortisol secretory response after stimulation with vasopressin.