A Rare Case of Cholestasis

Abstract

This is the case of 49-year-old woman with no past medical history who was found to have a rare case of cholangitis. A 49-year-old woman with no significant past medical history presented with a 1-month history of dull abdominal pain, early satiety, fatigue, pruritus, and weight loss. She had no history of drug, alcohol, or tobacco use, and no family history of hepatobiliary disease or autoimmunity. Her vital signs were within normal limits. Physical exam revealed mild jaundice without abdominal tenderness or ascites. Initial lab testing showed elevated liver enzymes in a cholestatic pattern, with AST/ALT 147/34 U/L, alkaline phosphatase 493U/L, and total bilirubin 6.8 mg/dl (direct 4.0 mg/dl). Imaging, including CT, ultrasound, and MRCP, was suggestive of mild inflammation of the pancreas and mild splenomegaly, as well as mild fatty infiltration of the liver without gallstones, ductal obstruction, or focal hepatic or gallbladder abnormalities. Infectious studies were negative for viral hepatitis and HIV. ESR and CRP were elevated concerning for autoimmune etiology. Anti-nuclear antibody (ANA, titer 1:1280) and anti-actin antibody were positive. Anti-mitochondrial antibody (AMA) titer was negative. Liver biopsy was performed and showed biliary pattern cirrhosis and florid biliary duct destruction consistent with autoimmune cholangitis. The patient was started on prednisone and ursodeoxycholic acid with resolution of symptoms 1 month later. AST was 45U/L, alkaline phosphatase 130U/L, and total bilirubin decreased to 1.2 mg/L. Autoimmune cholangitis (AIC) is a rare cause of cholestasis not commonly encountered in clinical practice. Maintaining it as a differential diagnosis will aid in prompt diagnosis and treatment. The patient met diagnostic criteria for AIC, which is characterized by the clinical, biochemical, and histologic features of primary biliary cirrhosis (PBC) but with negative AMA and positive ANA and/or anti-actin antibody. Like PBC, AIC has a female preponderance, a cholestatic liver enzyme pattern, and florid bile duct lesions on histology; both slowly progress to fibrosis and cirrhosis if left untreated. AIC is probably a heterogeneous syndrome that includes patients with AMA-negative PBC, small duct primary sclerosing cholangitis, autoimmune hepatitis with bile duct damage, and various transition states. Therapy consists of ursodeoxycholic acid and corticosteroids; liver transplantation is the treatment of choice for end-stage disease.