Published online Jun 7, 2018. doi: 10.3748/wjg.v24.i21.2320
Peer-review started: January 22, 2018
First decision: February 26, 2018
Revised: March 8, 2018
Accepted: March 31, 2018
Article in press: March 31, 2018
Published online: June 7, 2018
Processing time: 134 Days and 14.8 Hours
Bile acid diarrhea results from excessive amounts of bile acids entering the colon due to hepatic overexcretion of bile acids or bile acid malabsorption in the terminal ileum. The main therapies include bile acid sequestrants, such as colestyramine and colesevelam, which may be given in combination with the opioid receptor agonist loperamide. Some patients are refractory to conventional treatments. We report the use of the farnesoid X receptor agonist obeticholic acid in a patient with refractory bile acid diarrhea and subsequent intestinal failure. A 32-year-old woman with quiescent colonic Crohn’s disease and a normal terminal ileum had been diagnosed with severe bile acid malabsorption and complained of watery diarrhea and fatigue. The diarrhea resulted in hypokalemia and sodium depletion that made her dependent on twice weekly intravenous fluid and electrolyte infusions. Conventional therapies with colestyramine, colesevelam, and loperamide had no effect. Second-line antisecretory therapies with pantoprazole, liraglutide, and octreotide also failed. Third-line treatment with obeticholic acid reduced the number of stools from an average of 13 to an average of 7 per 24 h and improved the patient’s quality of life. The fluid and electrolyte balances normalized. The effect was sustained during follow-up for 6 mo with treatment at a daily dosage of 25 mg. The diarrhea worsened shortly after cessation of obeticholic acid. This case report supports the initial report that obeticholic acid may reduce bile acid production and improve symptoms in patients with bile acid diarrhea.
Core tip: Bile acid diarrhea develops when excessive amounts of bile acids enter the terminal ileum and exceed the intestinal absorptive capacity. The excess bile acids enter the colon and cause secretory diarrhea. We report a patient with multiple potential causes of chronic diarrhea and suggest a systematic strategy for the diagnosis and treatment of this condition. Furthermore, we describe the use of a new treatment for severe bile acid diarrhea, obeticholic acid, which stimulates the farnesoid X receptor of the terminal ileum and increases fibroblast growth factor 19, thereby decreasing hepatic bile acid production via negative feedback.