Cushing's disease.
Cushing's disease, or pituitary ACTH dependent Cushing's syndrome, is a rare disease responsible for increased morbidity and mortality. Signs and symptoms of hypercortisolism are usually non specific: obesity, signs of protein wasting, increased blood pressure, variable levels of hirsutism. Diagnosis is frequently difficult, and requires a strict algorithm. First-line treatment is based on transsphenoidal surgery, which cures 80% of ACTH-secreting microadenomas. The rate of remission is lower in macroadenomas. Other therapeutic modalities including anticortisolic drugs, radiation techniques or bilateral adrenalectomy will thus be necessary to avoid long-term risks (metabolic syndrome, osteoporosis, cardiovascular disease) of hypercortisolism. This review summarizes potential pathophysiological mechanisms, diagnostic approaches, and therapies.
Department of Endocrinology and Reference Center for Rare Pituitary Diseases La Timone Hospital, Aix-Marseille University, Marseille, France. Frederic.castinetti@ap-hm.fr
Frederic
F
Castinetti
Isabelle
I
Morange
Bernard
B
Conte-Devolx
Thierry
T
Brue
D049913:ACTH-Secreting Pituitary Adenoma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D006801:Humans; D047748:Pituitary ACTH Hypersecretion / Q000175:diagnosis / Q000473:pathology / Q000601:surgery / Q000628:therapy; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D035583:Rare Diseases