ACTH deficiency is a rare cause of secondary adrenocortical insufficiency and frequently associated autoimmune-mediated hypothyroidism, but there has been no report associated autoimmune-mediated hepatitis. We present a 36-year-old female with generalized weakness, malaise, hypersomnolence. Investigations showed low albumin, prolonged PT, elevated bilirubin level and abdominal ultrasonography and CT revealed liver cirrhosis with ascites. Though serologic test for hapatopathy were all negative and there was no alcoholic history, antibody for autoimmune-mediated hepatitis were positive such as smooth muscle antibody, SS-A/Ro antibody. Primary hypothyroidism was documented by TFT and Thyroid antibody such as antimicrosomal and TSH-R antibody were positive. There was no change on the patients weakness after thyroid hormone replacement, the pituitary-adrenal axis was examined. No abnormal findings such as tumor, or empty seller were found on a MRI of the pituitary gland. Dynamic test for pituitary hormones secretion in response to combined stimulation with CRH, TRH, LHRH and GRH revealed a blunted response of ACTH, exaggerated response of TSH and normal responses of prolactin, LH, FSH, GH. During rapid ACTH stimulation, secretion of cortisol were blunted while secretion of aldosterone were normal. After replacement with thyroxine and glucocorticoids, she gradually improved and discharged at the hospital. In conclusion, we report the case of isolated ACTH deficiency associated with autoimmune-mediated hypothyroidism and hepatitis.
01 - 05 Apr 2006
European Society of Endocrinology