DOI: 10.4103/ghep.ghep_2_26 ISSN: 2772-2732

Autoimmune Hepatitis-primary Biliary Cholangitis Overlap Syndrome: A Rare Cause of Jaundice

A. C. Arun, Jenish Rajma, Rithi S Preetha

Abstract

We report the case of a 43-year-old female with hypothyroidism and rheumatoid arthritis presenting with weakness and fatigue. Laboratory investigations revealed elevated transaminases (aspartate aminotransferase 233 IU/L and alanine aminotransferase 289 IU/L), alkaline phosphatase 385 IU/L, positive antinuclear antibodies (1:1280), smooth muscle antibody, and anti-mitochondrial antibody M2. Liver biopsy demonstrated interface hepatitis, florid ductopenia, and perivenular cholestasis fulfilling Paris criteria for autoimmune hepatitis-primary biliary cholangitis overlap syndrome. Treatment with ursodeoxycholic acid, prednisolone, and azathioprine led to sustained biochemical remission over 4 years. This case underscores the importance of identifying overlap syndrome in patients with autoimmune disorders.

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