Primary biliary cholangitis in Thai woman: A case report

Abstract

Primary biliary cholangitis or primary biliary cirrhosis (PBC) is a chronic, slowly progressive, autoimmune, cholestatic liver disease. It is globally considered a rare disease. Herein, we presented a 55-year-old Thai woman who gradually developed fatigue and generalized pruritis without fever for four months. Her physical examination revealed only pallor and jaundice, no liver stigmata, and no hepatosplenomegaly; her blood tests showed: Hb 8.1 g/dL, WBC 12,180/mm3, platelet 143,000/mm3, albumin 2.2 g/dL, globulin 4.0 g/dL, AST 164 U/L, ALT 102 U/L, alkaline phosphatase 384 U/L, total bilirubin 2.8 mg/dL, direct bilirubin 1.3 mg/dL, HBV, HCV and HIVnegative, ferritin 39.9 ng/ml, copper 125 ug/dL, ceruloplasmin 0.27 g/L, urine copper < 1 ug/g creatinine, antismooth muscle antibody-negative, anti-mitochondrial antibody-positive 1:1,600, ANA-positive, homogeneous pattern 1:80, centromere pattern 1:1,280, anti-cytoplasmic antibody-positive 1:1,280, alpha fetoprotein 3.16 ng/ml, creatinine 0.55 mg%, and normal coagulogram. Ultrasonography and the computerized tomography of the upper abdomen showed nodularity and heterogeneous parenchymal disease of the liver, no intrahepatic bile duct dilatation, and slight splenomegaly, compatible with cirrhosis. Esophagogastroduodenoscopy showed acute esophageal variceal bleeding that was successfully treated with rubber band ligation. The diagnosis of PBC was concluded and she was treated with ursodeoxycholic acid and propranolol. Within 6 months, her clinical symptoms as well as transaminitis partially improved.