Porto-Sinusoidal Vascular Disease: A New Nomenclature Different from Idiopathic Non-Cirrhotic Portal Hypertension.

Background And Aims: Porto-sinusoidal vascular disease (PSVD) as a novel clinical conception was modified on the basis of idiopathic non-cirrhotic portal hypertension (INCPH). This study aimed to compare the clinical, biochemical histological features and prognosis between the diagnostic criteria for PSVD and that of INCPH.

Methods: A total of 65 patients who underwent liver biopsies were analyzed retrospectively. The clinical, pathological and prognosis date were reviewed and screened according to the latest diagnostic criteria of PSVD and INCPH.

Results: A total of 65 patients were diagnosed with PSVD, of which 31 (47.69%) also fulfilled INCPH criteria. Specific histological and specific clinical portal hypertension (PH) signs were found in 34 (52.31%) and 30 (46.15%) of the patients, respectively. PSVD patients showed higher LSM levels (11.45 (6.38, 18.08) vs. 7.90 (6.70, 13.00), = 0.039) than the INCPH patients. INCPH patients had a higher cumulative incidence of liver-related complications than the PSVD patients (86.95% vs. 35.71%, log-rank < 0.001).

Conclusion: Novel PSVD criteria facilitate early diagnosis. PSVD patients with other liver diseases may have higher LSM values. Disease progression and survival outcomes are correlated with PH in PSVD patients.