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. The 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. The esophagogastroduodenoscopy showed acute
esophageal variceal bleeding that was successfully treated with the 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.