Expert consensus on the clinical management of pyrrolizidine alkaloid‐induced hepatic sinusoidal obstruction syndrome

Abstract

Hepatic sinusoidal obstruction syndrome (HSOS) is a hepatic vascular disease presenting with abdominal distension, pain in the hepatic region, ascites, jaundice, and hepatomegaly. In China, this disease is often associated with the oral intake of plants that contain pyrrolidine alkaloids. The existing guidelines are limited to HSOS associated with hematopoietic stem cell transplantation in Western countries. The Hepatobiliary Diseases Committee of the Chinese Society of Gastroenterology convened an expert consensus conference on the diagnosis and treatment of PA–HSOS to evaluate current research in China and abroad. The “Nanjing criteria” developed by the committee to diagnose PA–HSOS include a confirmed history of PA‐containing plant use and (i) abdominal distention and/or pain in the hepatic region, hepatomegaly, and ascites; (ii) elevation of serum total bilirubin or abnormal laboratory liver tests; (iii) evidence on enhanced computed tomography or magnetic resonance imaging; or (iv) pathological evidence that rules out other known causes of liver injury. Supportive symptomatic treatment, anticoagulant therapy, and placement of a transjugular intrahepatic portosystemic shunt for patients who do not respond to medical treatment are effective for the treatment of PA–HSOS. The benefits of glucocorticoids and prostaglandin E1 in PA–HSOS are not clear.

Clinical characteristics, CT signs, and pathological findings of Pyrrolizidine alkaloids-induced sinusoidal obstructive syndrome: a retrospective study

“…Then, dynamic enhancedcontrast CT scanning and/or MRI examination should be performed in all suspected patients. If typical signs of PA-induced HSOS are discovered, then the diagnosis can be confirmed [13,14,19,42,43]. Other chronic liver diseases, such as BCS, decompensated cirrhosis, cardiac insufficiency, should be excluded.…”

“…In guideline of pyrrolizidine alkaloid-induced HSOS issued by Chinese Society of Gastroenterology Committee, supportive symptomatic treatment is the basic PA-HSOS treatment regimen. Anticoagulant therapy should be started as soon as possible in acute/subacute stage patients after ruling out contraindications TIPS should be considered when the patients do not respond to medical treatment [42]. Public education programs on potential harms of PAs-containing plants should be developed through five media (newspapers, TV, the internet, radio, and magazines).…”

Low molecular weight heparin in the treatment of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome: five case reports

“… 12 However, in China, the main cause of HSOS is ingestion of PA-containing herbals. 13 Therefore, the Chinese Society of Gastroenterology Committee of Hepatobiliary has formulated specific diagnostic criteria (Nanjing Criteria) for this disease. 13 Together with a history of PA exposure, the patients must meet the following three conditions: (i) signs or symptoms of abdominal pain, abdominal distension, hepatomegaly and ascites; (ii) elevated total bilirubin or other liver function abnormalities; and (iii) imaging examination showing typical contrast-enhanced CT or magnetic resonance imaging manifestations, that is, the liver has ‘plaque-like’ and ‘map-like’ uneven density changes.…”

Retrospective analysis of the efficacy and safety of rivaroxaban in the treatment of hepatic sinus obstruction syndrome caused by Gynura segetum

“…Low molecular weight heparin in combination or sequence with warfarin are recommended as anticoagulant regimens in the "Nanjing criteria" [6] . Previous studies of heparin or low molecular weight heparin in the treatment of a small series of PA-HSOS patients in China reported recovery rates of 70.7%-88.9% [5] . Low molecular weight heparin is significantly safer than unfractionated heparin.…”

“…The classical triad of PA-HSOS consists of ascites, hepatomegaly, and increased bilirubin levels. The typical manifestations are swelling, damage and shedding of hepatic sinusoid endothelial cells in the hepatic acinus zone III and significant dilation and congestion of the hepatic sinusoids [5] developed by the committee to diagnose PA-HSOS include a confirmed history of PA-containing plant ingestion and the following: 1) abdominal distention and/or pain in the hepatic region, hepatomegaly and ascites, 2) elevation of serum total bilirubin levels or abnormal laboratory liver tests, and 3) evidence on enhanced CT or MRI, or pathological evidence that rules out other known causes of liver injury [6] . There is no specific treatment for PA-HSOS, and the mortality rate is high.…”