Triple overlap syndrome involving autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) is an exceptionally rare autoimmune hepatopathy, posing major diagnostic and therapeutic challenges. A 51-year-old woman initially diagnosed with AIH-PBC overlap presented persistent hepatocellular cytolysis despite corticosteroids and ursodeoxycholic acid. Autoimmune screening revealed ANA, anti-M2, and anti-gp210 positivity. Repeat liver biopsy showed interface hepatitis, ductopenic lymphocytic cholangitis, and periductal fibrosis. Magnetic resonance cholangiography demonstrated an attenuated intrahepatic biliary tree with a "pruned-tree" appearance, confirming PSC features. The diagnosis of AIH-PBC-PSC triple overlap was established. Due to azathioprine intolerance and hepatotoxicity, therapy was switched to mycophenolate mofetil with UDCA and low-dose corticosteroids, resulting in biochemical stabilization. This case represents one of the few well-documented instances of AIH-PBC-PSC overlap. It underscores the importance of integrating serology, histology, and advanced imaging for accurate diagnosis and individualized therapy. In the absence of standardized guidelines, management relies on tailored immunosuppression and supportive care to prevent progression toward liver failure.
Keywords: Autoimmune hepatitis; case reports; overlap syndrom; primary biliary cholangitis; primary sclerosing cholangitis.
This report discusses a very rare liver condition where three autoimmune diseases—AIH, PBC, and PSC—occur together. Because these diseases normally occur separately, having all three at once makes the diagnosis and treatment more difficult. We describe a 51-year-old woman whose tests, liver biopsy, and MRI scans showed signs of all three conditions. The standard treatments did not work well, so her doctors used a combination including corticosteroids, ursodeoxycholic acid, and mycophenolate mofetil. Her blood tests later stabilized. This case shows how important it is for doctors to combine different types of tests to reach the right diagnosis and to adapt treatment individually when guidelines do not exist.