Introduction: The clinical presentation of autoimmune hepatitis (AIH) is highly variable and provides a significant diagnostic challenge for physicians. We present a rare case of a young female with AIH-associated with peripheral eosinophilia. Case Report: A 24-year-old white female with an intellectual disability presented with a 2-week history of mild fever, new onset ascites, and leg swelling. Two months ago, she had an episode of acute hepatitis A with positive IgG and IgM antibodies. Physical examination revealed ascites, hepatomegaly, and pedal edema. The rest of her exam was unremarkable. Laboratory data showed leukocytosis (12.7) with eosinophilia (64%). Liver function tests were abnormal with bilirubin 1.4 mg/dL, albumin 2.8 g/dL, elevated globulin 4.7 g/dL, ALT 212 U/L, AST 234 U/L, ALP 209 U/L, and INR 1.53. Serum protein electrophoresis showed hypergammaglobulinemia. Stool examination and serology for parasites were negative. Hepatitis A IgG antibody remained reactive while IgM was now equivocal. HBsAg, anti-HCV, and serum acetaminophen were negative. ANA was positive. Total complement was low. CT abdomen showed hepatosplenomegaly, marked ascites, and abdominal wall edema. ECHO was unremarkable. Ascitic fluid analysis revealed a serum ascites albumin gradient of 2.8 and unremarkable cytology. Bone marrow was normocellular with iron deficiency, eosinophilia, and no increase in blasts. Cytogenetics and FISH were normal. Liver biopsy showed chronic hepatitis with moderate periportal infiltrate of small lymphocytes, plasma cells, few eosinophils, and extensive bridging fibrosis. Hence, a diagnosis of AIH was made based on her presentation, hypergammaglobulinemia, positive ANA, low complement, and a liver biopsy compatible with AIH. She was started on steroids, along with diuretics, with improvement in her symptoms. Her liver enzymes continued to improve and her eosinophil count normalized prior to discharge. Discussion: AIH is an inflammatory disease of liver with a female preponderance. The clinical presentation mainly includes features of chronic liver disease. It can, at times, be associated with other extra-hepatic autoimmune disorders and overlap syndromes not seen in our patient. The cause of peripheral eosinophilia in our patient is unclear. Very few cases of AIH associated with peripheral eosinophilia have been reported in literature. Hypereosinophilic syndrome was excluded by the lack of organ involvement. A significant percentage of patients with AIH develop cirrhosis, and 5% develop hepatocellular cancer. Hence, cancer surveillance is important in these patients. Treatment usually includes steroid therapy with or with out the use of other immunosuppressive therapy.
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