Endocrine Abstracts

Introduction: Cushing’s disease is a rare disorder caused by the hypersecretion of corticosteroids. Primary biliary cirrhosis (PBC) is a chronic autoimmune cholestatic hepatopathy characterized by destruction of the bile ducts and the presence of m²-type anti-mitchondrial antibodies. We report a rare association between cushing’s disease and primary biliary cirrhosis.

Observation: The patient was 38 years old, with recent history of diabetes and dyslipidemia, was admitted to our department of endocrinology for suspicion of a Cushing syndrome. The physical examination showed facial erythrosis, filling of the supra-scalvicular hollows, presence of a buffalo hump and facio-truncular fat distribution but not catabolic signs. Diagnosis of ACTH-dependent cushing’s syndrome biologically was made on the basis of lack of plasma cortisol response to low dexamethasone suppression test at 516 nmol/l (above 50 nmol/l), and high ACTH value at 69.4 confirmed by a second assessment at 100 pg/ml. High-dose Dexamethasone Suppression test cortisol level decrease greater than 50% after 8 mg of dexamethasone (from 728 to 315 nmol/l), suggesting Cushing disease. Pituitary MRI showed a 6 mm micro-adenoma. She underwent a transphenoidal adenomectomy with post operative remission. Moreover, the biological work -up showed cytolysis at 1.5 time the upper limit with cholestasis. Viral serologies and autoimmune antibodies were carried out, showing positive anti-mitochondrial m² ACs and negative viral serologies. Abdominal ultrasound revealed no abnormalities. A liver biopsy was performed, showing epithelioid granulomas with neoductular proliferation, confirming the diagnosis. The patient was treated by ursalvan,

Conclusion: Cushing’s syndrome has been associated in the literature to several liver diseases, hepatic steatosis being the most common. coexistance between cushing’s disease and primary biliary cirrhosis (PBC) is uncommon and has been little reported Common physiopathology must be investigated.