THU242 An Adolescent Female With Autoimmune Hepatitis And Graves’ Disease: An Unusual Presentation And Challenges In Management
Authors: Velasquez Horacio Javier , Ruiz Santillan Marco Andres , Paul David L , Tosur Mustafa 
Published: October 2023

Disclosure: H.J. Velasquez: None. M.A. Ruiz Santillan: None. D.L. Paul: None. M. Tosur: None.

Background: Presence of multiple autoimmune disorders in an individual is not uncommon. Simultaneous diagnoses of autoimmune hepatitis (AIH) and Graves’ disease in a previously healthy adolescent individual has not been previously reported. Here we report an unusual clinical presentation of both autoimmune conditions in an adolescent female. Case Presentation: A 13-year-old Hispanic female presented with jaundice, fatigue, and weight loss, was found to have hepatic failure. She tested positive for autoantibodies (Ab) associated with AIH (Kidney-Liver Microsome Ab &gt;1:2560, n &lt;1:20; IgG 3480 mg/dl, n 641-1353 mg/dl; Anti Smooth muscle Ab &lt;1:20, n &lt;1:20; ANA 1:1280, n &lt;1:160). Family history was negative for autoimmune diseases. During admission she remained with tachycardia (heart rate &gt;100 beats per minute) and elevated systolic blood pressure (&gt;120 mm Hg). Family confirmed tachycardia and mild exophthalmos started recently before admission. Due to concern for additional autoimmune diseases, thyroid function tests were performed and resulted abnormal (TSH &lt;0.010 uIU/ml, n 0.7-4.1 uIU/ml; Free T4 4.7 ng/dl, n 0.8-2.0 ng/dl; Free T3 1023 pg/dl, n 286-556 pg/dl; total T3 199 ng/dl, n 80-210). Further autoantibody tests were consistent with Graves’ disease (TSI Ab 7.67IU/L, n&lt;=0.54), other thyroid antibodies were negative (Thyroid Peroxidase Ab &lt; 3 IU/ml, n&lt;= 5.6 IU/L and Anti-Thyroglobulin Ab &lt; 3 IU/ml, n&lt;= 4 IU/L). Due to liver failure, antithyroid medications were contraindicated. Pending liver biopsy to confirm AIH made steroids not an option due to risk of alteration in biopsy results. The patient was treated with propranolol alone. Once her thyroid hormone levels stabilized, she had liver biopsy and then was placed on a steroid regimen. Her hospitalization was complicated by decompensated cirrhosis with hyperammonemia (ammonia 180 umol/l, n 22-48 umol/l), hepatic encephalopathy and hemodynamically unstable requiring intubation and ICU transfer. Hyperammonemia responded well to rifaximin and lactulose. After clinical improvement, she was discharged home with high dose prednisone, propranolol, ursodiol, phytonadione, lactulose, rifaximin, and spironolactone. Total thyroidectomy was performed two months later for permanent treatment of Grave’s disease. She was evaluated for liver transplantation and approved for listing but given her clinical improvement, she was listed as inactive status. Conclusions: AIH and Graves’ disease can present simultaneously. Screening for thyroid disorders should be considered when AIH is suspected. Co-presence of AIH makes acute management of Graves’ disease particularly challenging. An individualized treatment approach should be implemented after carefully reviewing risk/benefit ratios of each treatment option.

Presentation: Thursday, June 15, 2023