Additional studies are being required to prove its clinical benefit. Primary biliary cholangitis
Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is an auto-immune, inflammatory liver disease which produces bile duct injury, fibrosis, cholestasis and eventual cirrhosis. It is much more common in women than men and can cause jaundice, itching (pruritus) and fatigue. Ursodeoxycholic acid therapy is beneficial, but the disease often progresses and may require liver transplantation. Animal studies suggested that treatment with FXR agonists should be beneficial in cholestatic diseases such as PBC. OCA at doses between 10 mg and 50 mg was shown to provide significant biochemical benefit, but pruritus was more frequent with higher doses. The results of a randomized, double-blind phase III study of OCA, 5 mg or 10 mg, compared to placebo (POISE) were presented in April 2014, and showed that the drug met the trials primary endpoint of a significant reduction in serum alkaline phosphatase, a biomarker predictive of disease progression, liver transplantation or death. Nonalcoholic steatohepatitis (NASH)
Non-alcoholic steatohepatitis is a common cause of abnormal liver function with histological features of fatty liver,  inflammation and fibrosis. It may progress to cirrhosis and is becoming an increasing indication for liver transplantation. It is increasing in prevalence. OCA is proposed to treat NASH.
Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis, is a chronic infection characterized by recurrent bouts of bacterial cholangitis with primary hepatolithiasis. It is exclusive to people who live or have lived in southeast Asia. Presentation
Presentation can be atypical and without pain or fever, especially in the elderly. Positive symptoms include biliary colic, acute pancreatitis, obstructive jaundice and less commonly, liver enlargement and abnormal liver function tests. Additional complications in the acute setting include ascending cholangitis, gallbladder empyema, clotting within the hepatic and portal veins, sepsis and death.Chronic biliary obstruction may cause jaundice, itchiness, liver abscesses, and cirrhosis, particularly at the left lobe segment 3, and can eventually lead to intraductal papillary mucinous neoplasm or cholangiocarcinoma. Pathogenesis
With RPC, the gallstones found within the biliary system are made of calcium bilirubinate or pigmented calcium. Calcium bilirubinate stones are prevalent in Asia and very rare in Europe and the United States. They tend to be friable concretions of various shapes and sizes within the biliary tree, and their associated bile is often muddy in consistency and contains numerous fine particles of calcium bilirubinate as well. This differs greatly from cholesterol stones, which are common in Europe and the United States. The formation of calcium bilirubinate stones in RPC has been attributed to a high incidence of infection with Escherichia coli in bile. In humans, the majority of bilirubin is excreted in the bile as bilirubin glucuronide.Hepatolithiasis is associated with Clonorchis sinensis and Ascaris lumbricoides infestation of the liver.