Adrenal Cushing's syndrome due to bilateral macronodular adrenal hyperplasia: prediction of the efficacy of beta-blockade therapy and interest of unilateral adrenalectomy.
Bilateral adrenalectomy is the standard treatment for Cushing's syndrome (CS) related to ACTH-independent bilateral macronodular hyperplasia (AIMAH), although it imposes life-long adrenal insufficiency. This study reports a clinical case in order to discuss the clinical interest of pharmacological beta-blockade of illegitimate membrane receptors and unilateral adrenalectomy as alternatives to bilateral adrenalectomy for treatment of CS due to AIMAH. Evidence for cortisol stimulation by upright posture and insulin-induced hypoglycemia in a patient with CS related to AIMAH led us to try beta-blockers as a therapeutic test and then as a first line treatment. Thus, a 3-day beta-blocker test (320 mg/d propranolol) induced normalization of cortisol secretion, with return of hypercortisolism at the end of the test. A long term treatment with 320 mg/d propranolol allowed sustained normalization of cortisol secretion and progressive disappearance of Cushingoid features but after 8 months the patient complained of Raynaud's syndrome and fatigue. Lowering propranolol dosage or switching to atenolol was less efficient to reduce cortisol levels. Unilateral adrenalectomy was then performed as a second line treatment, leading to normalisation of the 24h urinary cortisol without adrenal insufficiency. Long term control of blood pressure and glycemia were observed during a 7-year follow-up without beta-blocker. In conclusion, a 3-day propranolol test may identify patients with AIMAH who can benefit from a long term beta-blocker treatment. In case of intolerance to beta-blocking agents, unilateral adrenalectomy may allow for long term control of Cushing's syndrome related to AIMAH without adrenal insufficiency.
Mazzuco
Tânia L
TL
Service d'Endocrinologie, Centre Hospitalier Universitaire A. Michallon, Grenoble, France.
Chaffanjon
Philippe
P
Martinie
Monique
M
Sturm
Nathalie
N
Chabre
Olivier
O
D000311:Adrenal Glands / Q000473:pathology*; D000315:Adrenalectomy / Q000379:methods*; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D001262:Atenolol; D003480:Cushing Syndrome / Q000188:drug therapy* / Q000601:surgery*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000652:urine; D006965:Hyperplasia; D008875:Middle Aged; D011187:Posture; D011433:Propranolol / Q000627:therapeutic use*; D016896:Treatment Outcome; D014667:Vasopressins / Q000097:blood
D000319:Adrenergic beta-Antagonists; D014667:Vasopressins; D001262:Atenolol; D011433:Propranolol; D006854:Hydrocortisone