A case of asymptomatic non-cirrhotic primary biliary cholangitis showing splenorenal shunt with neither anti-mitochondrial antibody nor M2 antibody

Abstract

We report a case of asymptomatic non-cirrhotic primary biliary cholangitis (PBC) showing splenorenal shunt with neither anti-mitochondrial antibody nor M2 antibody. A 67-year-old female with Sjögren’s syndrome was admitted to our hospital to undergo liver biopsy and hepatic venous catheterization for detailed evaluation of liver disease because of the elevation of serum ammonia level and to-and-fro appearance in the splenic vein. There were no clinical signs of encephalopathy and itching. Abdominal computed tomography showed no findings for chronic liver disease, no evidence of splenomegaly or ascites. Hepatic venography showed no abnormal findings in the hepatic vein and intrahepatic portal vein, and hepatic venous pressure gradient was 4.8 mmHg. Final diagnosis was made by liver biopsy showing chronic non-suppurative destructive cholangitis, indicating PBC (Scheuer’s classification, stage 2; Nakanuma’s classification, stage 3).