Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 7, 2018; 24(21): 2320-2326
Published online Jun 7, 2018. doi: 10.3748/wjg.v24.i21.2320
Obeticholic acid for severe bile acid diarrhea with intestinal failure: A case report and review of the literature
Christian Lodberg Hvas, Peter Ott, Peter Paine, Simon Lal, Søren Peter Jørgensen, Jens Frederik Dahlerup
Christian Lodberg Hvas, Peter Ott, Søren Peter Jørgensen, Jens Frederik Dahlerup, Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus C 8000, Denmark
Peter Paine, Simon Lal, Department of Gastroenterology, Salford Royal NHS Foundation Trust, Salford, Manchester M6 8HD, United Kingdom
Author contributions: Hvas CL treated the intestinal failure in the patient and wrote the first draft of the manuscript; Ott P managed the communications with Intercept Pharmaceuticals, discussed the treatment and drafted the manuscript; Paine P and Lal S discussed the differential diagnoses during the treatment of the patient and revised the manuscript; Jørgensen SP provided expertise on BAD and FXR biology and revised the manuscript; Dahlerup JF was responsible for treating the patient, handled the communication with National Health Authorities, and revised the manuscript; all authors approved the final version of the manuscript.
Informed consent statement: The patient gave oral and written consent for the publication of this case report. A signed informed consent statement has been uploaded with the submission of the manuscript.
Conflict-of-interest statement: Hvas CL reports lecture fees from Takeda, Ferring, Novartis, MSD, and Tillotts. Ott P reports a lecture fee from Intercept Pharmaceuticals. Lal S reports grants from Fresenius Kabi and Shire. Dahlerup JF reports lecture fees from Pharmacosmos, MSD, and Takeda. All other authors declare that they have no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Christian Lodberg Hvas, MD, PhD, Associate Professor, Department of Hepatology and Gastroenterology, Aarhus University Hospital, Nørrebrogade 44, Aarhus C 8000, Denmark.
Telephone: +45-78463895 Fax: +45-78462820
Received: January 20, 2018
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
Case characteristics

A 32-year-old woman with chronic diarrhea that had multiple potential causes including bile acid diarrhea, Crohn’s disease, and medications for epilepsy and depression.

Clinical diagnosis

Bile acid diarrhea (BAD), diagnosed by selenium homotaurocholic acid test scintigraphy with 0 bile acid retention after seven days.

Laboratory diagnosis

Persistently low plasma levels of sodium and potassium and undetectable 24-h urine sodium excretion, indicating intestinal failure with dependency of intravenous fluid support.

Pathological diagnosis

Normal duodenal, jejunal, ileal, and colonic biopsies, indicating quiescent Crohn’s disease. Positive Clostridium difficile toxin PCR test indicating Clostridium difficile colitis.


Clostridium difficile colitis was treated with vancomycin followed by fecal microbiota transplantation. Bile acid diarrhea was refractory to conventional treatments including colestyramine and colesevelam, and oral obeticholic acid treatment was commenced at 10 mg per day, increasing to 25 mg per day. Upon this, the patient’s bowel habits and quality of life improved.

Related reports

Obeticholic is licensed for primary biliary cholangitis and has been used in non-alcoholic steatohepatitis. It was recently reported that obeticholic acid may improve bile acid diarrhea through induction of fibroblast growth factor 19 that inhibits hepatic bile production.

Term explanation

BAD–bile acid diarrhea, resulting from excess hepatic production and/or deficient ileal reabsorption of bile acids, which in turn induces colonic fluid and electrolyte secretion and leads to chronic secretory diarrhea.

Experiences and lessons

In patients with chronic diarrhea, a thorough and systematic diagnostic workup may help to differentiate between potential causes of diarrhea. Some patients with bile acid diarrhea are refractory to conventional treatments. Obeticholic acid may be of clinical benefit in these patients.