Abstract

This report discusses a 53-year-old female who complained of jaundice, right upper quadrant abdominal pain, joint complaints, swelling, weight loss, and a family history of similar disease. Laboratory findings revealed marked elevations in ALT (125 IU/L), AST (430 IU/L), Gamma GT (1051.2 IU/L), and ALP (161 IU/L), along with cholestasis (total bilirubin 6.35 mg/dL, direct bilirubin 5.76 mg/dL), and hypoalbuminemia (2.57 g/dL). Serology revealed positive anti-smooth muscle antibody (ASMA) titers and anti-mitochondrial antibody (AMA) (≥1:20), increased antinuclear antibody (ANA) levels (81.3 IU), decreased Complement C3 (76.0; range, 82–185), and low-normal Complement C4 (17.3; range, 15–53). Coomb’s test was directly positive. MRCP demonstrated cholangitis and narrowing of the cystic duct, likely secondary to mucosal oedema, and liver biopsy confirmed chronic hepatitis with mild fibrosis (F2). Extrahepatic cholestasis was highlighted by immunohistochemistry. We made the diagnosis of overlap syndrome (AIH-PBC-PSC), and the patient received ursodeoxycholic acid, n-acetylcysteine, methylprednisolone, hydroxychloroquine, mycophenolic acid, and calcium supplements. Clinical and laboratory improvement was noted post-treatment, with normalization of laboratory parameters

Key words: Overlap syndrome, autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, steroids.