Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome With Limited Systemic Sclerosis

Autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome is an uncommon autoimmune liver disease, and its association with limited systemic sclerosis is rarely reported. We describe the case of a 41-year-old woman with a history of intrahepatic cholestasis of pregnancy (ICP) who presented with chronic pruritus, arthralgias, recurrent urinary tract infections, and Raynaud phenomenon. Laboratory evaluation demonstrated a predominantly cholestatic liver profile, positive antimitochondrial antibodies (AMAs), high-titer antinuclear antibodies (ANA) with a centromere pattern, elevated immunoglobulin G (IgG), and negative viral serologies. Imaging revealed cirrhotic liver morphology, splenomegaly, and features of clinically significant portal hypertension. Liver biopsy showed interface hepatitis with lymphoplasmacytic infiltrates, florid duct lesions, and ductopenia, supporting the diagnosis of AIH-PBC overlap syndrome in conjunction with the clinical and serological findings. Positive anticentromere antibodies confirmed limited systemic sclerosis. Despite treatment with ursodeoxycholic acid and immunosuppressive therapy, the patient experienced progressive liver dysfunction, developing decompensated cirrhosis with ascites and worsening portal hypertension, and is currently undergoing liver transplant evaluation. This case highlights the importance of recognizing extrahepatic autoimmune manifestations and integrating clinical, serological, and histopathological findings to establish an early diagnosis of AIH-PBC overlap syndrome associated with limited systemic sclerosis, allowing timely treatment and referral for liver transplantation in patients with progressive disease.