Transjugular intrahepatic portosystemic shunt improves transplant-free survival of PA-HSOS patients with no response to anticoagulation therapy——A multicenter retrospective study

Background & Aims Anticoagulation therapy is recommended during the acute or subacute stage for patients with pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome (PAHSOS). Transjugular intrahepatic portosystemic shunt (TIPS) is suggested as step-up treatment when patients have no response to anticoagulants. However, the efficacy of TIPS in patients who do not respond to anticoagulants needs more evidence. The aim of this study was to evaluate the effect of TIPS in PA-HSOS patients with no response to initial anticoagulation treatment. Methods We retrospectively enrolled PA-HSOS patients diagnosed based on the ‘Nanjing criteria’ between January 2013 and September 2020 from four hospitals, and all of them had no response to initial short-term anticoagulation therapy. Then, depending on the next treatment option, these patients were divided into the TIPS treatment group and the anticoagulation therapy group. The baseline information and clinical characteristics were collected and recorded. Liver transplant-free survival in both groups was the primary study endpoint and the risk factors for patient death were further analyzed. Results A total of 473 PA-HSOS patients from four hospitals were included in the study, and 99 patients were enrolled according to the inclusion and exclusion criteria. Of these, 63 patients were in the TIPS group and 36 were in the anticoagulation therapy group. In the anticoagulation therapy group, 25 patients received continuous anticoagulation treatment until the end of the study, and 11 patients did not respond to the prolonged anticoagulation therapy and turned to TIPS therapy halfway through treatment. There were 17 deaths during the median follow-up time of 32.5 months. The different choices of treatment (TIPS or continuous anticoagulation therapy), age, aspartate aminotransferase (AST), and serum total bilirubin (STB) were independent risk factors for predicting death. The patients in the TIPS group had a significantly higher transplant-free survival than those in the continuous anticoagulation therapy group (P=0.028). The prognosis of the mild and moderate patients treated with TIPS was better than that of the severe patients according to Drum Tower Severity Scoring (DTSS), a novel PA-HSOS severity grading criteria, while the severe patients did not show significant benefit with TIPS treatment. Conclusions TIPS treatment can improve the transplant-free survival rate in PA-HSOS patients who have failed short-term anticoagulation therapy, and patients with DTSS of mild and moderate grade could benefit from TIPS.