Porto-sinusoidal vascular disorder with known etiologies had more severe portal hypertension and poorer outcomes

Lin Ma; Guanhua Zhang; Yu Wang; Min Li; Fuliang He; Lijuan Feng; Min Wang; Jimin Liu; Xinyan Zhao; Jidong Jia

doi:10.1016/j.dld.2024.10.025

Porto-sinusoidal vascular disorder with known etiologies had more severe portal hypertension and poorer outcomes

Dig Liver Dis. 2024 Nov 23:S1590-8658(24)01069-7. doi: 10.1016/j.dld.2024.10.025. Online ahead of print.

Authors

Lin Ma¹, Guanhua Zhang², Yu Wang³, Min Li⁴, Fuliang He⁵, Lijuan Feng⁶, Min Wang⁷, Jimin Liu⁸, Xinyan Zhao⁹, Jidong Jia¹⁰

Affiliations

¹ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
² Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
³ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
⁴ Department of Clinical Epidemiology and EBM, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China. Electronic address: [email protected].
⁵ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
⁶ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
⁷ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].
⁸ Department of Pathology and Molecular Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Canada. Electronic address: [email protected].
⁹ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China. Electronic address: [email protected].
¹⁰ Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China. Electronic address: [email protected].

PMID: 39581837
DOI: 10.1016/j.dld.2024.10.025

Abstract

Background: Porto-sinusoidal vascular disorder (PSVD) is a clinicopathological entity and often associated with various etiologies. We aimed to compare the clinical and pathological features and outcomes of PSVD in patients with and without known etiologies in a Chinese cohort.

Methods: This retrospective study enrolled liver-biopsy confirmed patients with PSVD. Patients' demographics, comorbidities, laboratory and imaging information were collected and the outcomes were followed up.

Results: Totally 128 patients with PSVD were included, with 75 (58.6 %) having known etiologies including prothrombotic conditions, immunological disorders, hematological disorders, drug exposure. PSVD patients with known etiologies exhibited significantly higher rates of gastroesophageal varices (87.0 % vs 54.0 %, p < 0.001), portosystemic collaterals (94.0 % vs. 58.0 %, p < 0.001), lower level of serum albumin (36.5 vs 40.0, p < 0.001), and a higher prevalence of portal vein thrombosis (PVT, 34.0 % vs 13.0 %, p = 0.010). During follow-up, PSVD patients with known etiologies had a significantly higher incidence of liver-related events (ascites, variceal hemorrhage or HE) (p = 0.027).

Conclusions: PSVD with known etiologies had more severe portal hypertension, poorer liver function reserve, higher PVT prevalence, and worse clinical outcomes.

Keywords: Hepatic decompensation; Portal hypertension; Portal vein thrombosis; Porto-sinusoidal vascular disorder; Survival.