Graves' disease with autoimmune hepatitis and primary Sclerosing cholangitis: an overlap syndrome

Autoimmune hepatitis (AIH) is a chronic inflammatory disease that results from autoantibody mediated hepatocyte injury. Given its immune-mediated mechanism, it is more likely to present with other autoimmune conditions, particularly involving thyroid gland. In patients presenting with both Graves' disease and hepatitis, the diagnostic challenge is to determine the cause of elevated liver enzymes. We present an 11-year-old girl with acute liver cell failure and cholestasis in a setting of untreated hyperthyroidism. She was diagnosed to have Graves' disease with AIH and primary sclerosing cholangitis. She underwent radioactive iodine ablation of thyroid gland and is currently responding well on steroid, azathioprine, and thyroid replacement therapy.