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

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.

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