P3C021: COVID and the Color Yellow: A pediatric Case of Autoimmune Hepatitis with Hepatic Fibrosis worsened by COVID 19 Infection

Introduction: Hepatic involvement is common in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, with abnormal liver biochemistries occurring in adult and pediatric patients. We report a rare pediatric case of autoimmune hepatitis with hepatic fibrosis worsened by a symptomatic COVID-19 infection.

Case Description: A 3-year-old female with a medical history significant for autism spectrum disorder presented with jaundice, scleral icterus, and dark urine for 2 days. She was admitted one week prior with fever, cough, rhinorrhea, and congestion. She had an acute COVID 19 infection and cholestatic hepatitis with elevated aspartate aminotransferase (AST) 614, alanine aminotransferase (ALT) 486, total bilirubin (TB) 4.1, and conjugated bilirubin (CB) 2.8. Vitamin K was administered for 3 days for International normalized ratio (INR) of 1.44. Transaminases improved and she had a normal abdominal ultrasound with Doppler. One week later she returned with brown-tinged urine and icterus. Physical exam was notable for icterus, diffuse jaundice, and hepatomegaly. Labs worsened including a TB 5.7, CB 4.3, AST 718, ALT 416 and INR 1.56 with persistent COVID positivity. She was given 5 more days of Vitamin K. Blood and urine cultures, thyroid studies, ceruloplasmin, iron studies, creatine kinase, and triglycerides were normal. She had a negative ANA but elevated anti-liver-kidney-microsomal antibody (anti-LKM) titer. Pathology results were consistent with autoimmune hepatitis with stage 3 fibrosis. She was started on IV methylprednisolone and discharged on prednisolone as transaminases improved with an AST 146, ALT 221, and INR 1.40. She completed a steroid taper and started azathioprine after thiopurine methyltransferase genotyping resulted normal. At 4-week follow-up, her labs normalized.

Discussion: SARS-CoV-2 infection can cause multisystem disease with involvement of respiratory, gastrointestinal, hepatic, and renal systems due to viral cell entry via angiotensin converting enzyme 2 receptor. SARS-CoV-2 has also been associated with worsening autoimmune diseases. Rare complications in children include MIS-C (persistent fever plus elevated inflammatory markers and multiorgan involvement), which our patient did not have. Autoimmune hepatitis is a chronic hepatitis characterized by immunologic features, including elevated autoantibodies and serum gamma globulin. Type 1 Autoimmune hepatitis, in adults and adolescents, is associated with antinuclear antibodies and/or anti-smooth muscle antibodies. Type 2 Autoimmune hepatitis, in infants and children, is characterized by antibodies to LKM and/or liver cytosol antigen.

Conclusion: Several studies have postulated SARS-CoV-2 infections cause hepatic injury leading to permanent damage and cirrhosis. In our patient, it is likely that SARS-CoV-2 led to an unmasking and decompensation of pre-existing Autoimmune hepatitis given the presence of fibrosis. This case outlines a rare but important relationship between SARS-COV-2 and Autoimmune Hepatitis. Clinician diligence is needed to evaluate underlying causes of hepatic dysfunction associated with SARS-CoV-2. SARS-CoV-2 vaccination has been at the forefront of public health as its widespread distribution is important in preventing SARS-CoV-2-related complications.