Heyerick L, Dhondt A, Van Vlierberghe H, Verhelst X, Raevens S, Geerts A. Early plasmapheresis in type 2 benign recurrent intrahepatic cholestasis: A case report and review of literature. World J Hepatol 2025; 17(2): 102375 [DOI: 10.4254/wjh.v17.i2.102375]
Corresponding Author of This Article
Lander Heyerick, MD, Department of Gastroenterology and Hepatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent 9000, Belgium. lander.heyerick@uzgent.be
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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: http://creativecommons.org/licenses/by-nc/4.0/
World J Hepatol. Feb 27, 2025; 17(2): 102375 Published online Feb 27, 2025. doi: 10.4254/wjh.v17.i2.102375
Early plasmapheresis in type 2 benign recurrent intrahepatic cholestasis: A case report and review of literature
Lander Heyerick, Annemieke Dhondt, Hans Van Vlierberghe, Xavier Verhelst, Sarah Raevens, Anja Geerts
Lander Heyerick, Hans Van Vlierberghe, Xavier Verhelst, Sarah Raevens, Anja Geerts, Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent 9000, Belgium
Annemieke Dhondt, Department of Nephrology, Ghent University Hospital, Ghent 9000, Belgium
Author contributions: Heyerick L drafted the manuscript; Heyerick L, Dhondt A and Geerts A were responsible for the clinical follow-up and collected the data of the patient presented in this case report; Heyerick L, Dhondt A, Van Vlierberghe H, Verhelst X, Raevens S and Geerts A analysed and interpreted the data; all of the authors read and approved the final version of the manuscript to be published.
Informed consent statement: This study was approved by the local Ethical Commission of Ghent University Hospital (No. CR-2023-0006). The patient provided written informed consent for publication of this report.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Lander Heyerick, MD, Department of Gastroenterology and Hepatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent 9000, Belgium. lander.heyerick@uzgent.be
Received: October 15, 2024 Revised: December 6, 2024 Accepted: January 7, 2025 Published online: February 27, 2025 Processing time: 127 Days and 12.6 Hours
Abstract
BACKGROUND
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive liver disease, causing episodic cholestasis with intense pruritus. This case report highlights the effectiveness of early plasmapheresis as a therapeutic option for BRIC type 2, offering rapid symptom relief and early termination of cholestatic episodes. It contributes to the limited evidence supporting plasmapheresis as a treatment for BRIC flares resistant to conventional therapies.
CASE SUMMARY
A 43-year-old male with BRIC type 2 presented with fatigue, jaundice, and severe pruritus, triggered by a recent mild severe acute respiratory syndrome coronavirus 2 infection. Laboratory results confirmed cholestasis with elevated bilirubin and alkaline phosphatase. First-line pharmacological treatments, including cholestyramine and rifampicin, failed. Endoscopic nasobiliary drainage was ineffective, prompting initiation of plasmapheresis. This intervention rapidly relieved pruritus, with complete biochemical normalisation after 11 sessions. Two years later, a similar episode occurred, and early reinitiation of plasmapheresis led to symptom resolution within two sessions and biochemical recovery within two weeks. The patient tolerated the procedure well, with no adverse effects observed. Follow-up showed no signs of cholestasis recurrence.
CONCLUSION
Plasmapheresis is a safe and effective option for therapy-refractory BRIC type 2, particularly when initiated early in cholestasis.
Core Tip: This case report highlights the successful use of early plasmapheresis in managing a patient with pruritus due to a cholestatic episode of benign recurrent intrahepatic cholestasis type 2, triggered by severe acute respiratory syndrome coronavirus 2 infection. Plasmapheresis provided rapid relief from severe pruritus when first-line therapies failed and, when reinitiated early during a consequent flare, significantly accelerated recovery from cholestasis. While the procedure’s invasiveness and associated costs underscore the importance of careful patient selection, prompt initiation should be considered in therapy-refractory cases to optimize outcomes.
