Endocrine Abstracts

Cushing disease is a rare endocrine disorder characterised by excess production of adrenocorticotropic hormone (ACTH) from a pituitary adenoma causing an excessive stimulation of the adrenal glands resulting in hypercortisolism. Most common clinical presentation includes a combination of signs like central obesity, facial plethora,ecchymosis, purple skin striae, hirsutism, acne, muscle weakness and atrophy. Hypertension, glucose intolerance and diabetes, hypokalaemia and osteoporosis are usual consequences of persistent uncontrolled hypercortisolism. Osteoporosis is a frequent pathology in elderly subjects mostly in menopaused women. It can be primary or, less frequently, secondary to another pathology. However, in male subjects, secondary osteoporosis is more frequent. We present the case of a 61-year-old male patient primary admitted for multiple vertebral fractures (T9, T10, T12, L1, L2, L3, L4, L5) requiring cementoplasty initially thought to be post traumatic (mild fall from his height) until a new fracture appeared without any trauma. Spinal MRI showed signs of osteoporosis and bone densitometry confirmed it with a T score L2-L4 of -4,5. Clinical re-evaluation during hospitalisation found very discrete signs of hypercortisolism: relative muscle weakness and atrophy, mild facial erythrosis and few bruising on the arms and legs. BMI was strictly normal as was the blood pressure. Biological analysis revealed increased 8 am cortisol and ACTH with loss on nictemeral secretion rhythm, 24h urinary cortisol up to six times the normal rate, no suppression after 1mg dexamethasone overnight suppression test, moderate hyperprolactinemia, hypogonadotropic hypogonadism. Blood glucose and potassium were in the normal range. Pituitary MRI revealed a macro adenoma with a slight expansion towards the left cavernous sinus and extension towards the sphenoidal sinus. Trans-sphenoidal surgery was performed after a 4 weeks Ketoconazole treatment. Pathological analysis confirmed a pituitary adenoma of 17×24×19 mm with an estimated Ki 67 of 15%. One month post-surgery evaluation was consistent with high afternoon and midnight cortisol rate, normalization of 24h urinary cortisol and non-suppression after 1-mg overnight dexamethasone. Therefore, hypercortisolism is one of the possible causes to look for when diagnosing a severe osteoporosis especially in a male subject.The particularity of this case is the revelation of a Cushing disease by severe osteoporotic complications, without any metabolic abnormalities and a very poor clinical presentation.

Keywords: Cushing disease, hypercortisolism, osteoporosis