Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 28, 2019; 25(28): 3753-3763
Published online Jul 28, 2019. doi: 10.3748/wjg.v25.i28.3753
Pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome: Pathogenesis, clinical manifestations, diagnosis, treatment, and outcomes
Xiao-Qian Yang, Jin Ye, Xin Li, Qian Li, Yu-Hu Song
Xiao-Qian Yang, Jin Ye, Yu-Hu Song, Division of Gastroenterology, Union Hospital, Tongji Medical college, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
Xin Li, Qian Li, Department of Radiology, Union Hospital, Tongji Medical college, Huazhong University of Science and technology, Wuhan 430022, Hubei Province, China
Author contributions: Yang XQ and Song YH reviewed the literature; Song YH and Ye J established the design and conception of the paper; Li X and Li Q provided the radiological imaging data; all authors contributed to writing and revision of this review.
Supported by National Natural Science Foundation of China, No. 81570555 and No. 81770582.
Conflict-of-interest statement: The authors declare no conflicts of interest.
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 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:
Corresponding author: Yu-Hu Song, MD, PhD, Chief Doctor, Professor, Division of Gastroenterology, Union Hospital, Tongji Medical college, Huazhong University of Science and Technology, No 1277, Jiefang Avenue, Wuhan 430022, Hubei Province, China.
Telephone: +86-27-85726678 Fax: +86-27-85726057
Received: March 17, 2019
Peer-review started: March 18, 2019
First decision: May 24, 2019
Revised: June 5, 2019
Accepted: June 25, 2019
Article in press: June 26, 2019
Published online: July 28, 2019

Hepatic sinusoidal obstruction syndrome (HSOS) can be caused by the intake of pyrrolizidine alkaloids (PAs). To date, PAs-induced HSOS has not been extensively studied. In view of the difference in etiology of HSOS between the West and China, clinical profiles, imaging findings, treatment, and outcomes of HSOS associated with hematopoietic stem cell transplantation or oxaliplatin might be hardly extrapolated to PAs-induced HSOS. Reactive metabolites derived from PAs form pyrrole-protein adducts that result in toxic destruction of hepatic sinusoidal endothelial cells. PAs-induced HSOS typically manifests as painful hepatomegaly, ascites, and jaundice. Laboratory tests revealed abnormal liver function tests were observed in most of the patients with PAs-induced HSOS. In addition, contrast computed tomography and magnetic resonance imaging scan show that patients with PAs-induced HSOS have distinct imaging features, which reveal that radiological imaging provides an effective noninvasive method for the diagnosis of PAs-induced HSOS. Liver biopsy and histological examination showed that PAs-induced HSOS displayed distinct features in acute and chronic stages. Therapeutic strategies for PAs-induced HSOS include rigorous fluid management, anticoagulant therapy, glucocorticoids, transjugular intrahepatic portosystemic shunt, liver transplantation, etc. The aim of this review is to describe the pathogenesis, clinical profiles, diagnostic criteria, treatment, and outcomes of PAs-induced HSOS.

Keywords: Hepatic sinusoidal obstruction syndrome, Pyrrolizidine alkaloids, Hepatic sinusoidal endothelial cells, Pyrrole-protein adducts, Diagnostic criteria, Symptomatic treatment, Anticoagulant therapy

Core tip: Hepatic sinusoidal obstruction syndrome (HSOS), also named hepatic veno-occlusive disease, is a hepatic vascular disease presenting with abdominal distension, painful hepatomegaly, jaundice, and weight gain. The intake of pyrrolizidine alkaloids (PAs) is one of the major etiologies of HSOS in China. Unfortunately, PAs-induced HSOS has not been extensively studied up to now. Here, we describe the pathogenesis, clinical profiles, diagnosis, treatment, and outcomes of patients with PAs-induced HSOS.