Clinical Course of Porto-Sinusoidal Vascular Disease Is Distinct From Idiopathic Noncirrhotic Portal Hypertension
| Autor: | Judith Stift, Albert Friedrich Stättermayer, Matthias Pinter, Thomas Reiberger, Mattias Mandorfer, Katharina Lampichler, Theresa Bucsics, Philipp Schwabl, Katharina Pomej, Bernhard Scheiner, Katharina Wöran, Teresa Binter, Benedikt Simbrunner, Rafael Paternostro, Michael Trauner, Georg Semmler, David Bauer, Mathias Jachs |
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| Rok vydání: | 2022 |
| Předmět: |
Liver Cirrhosis
medicine.medical_specialty Cirrhosis Biopsy Portal venous pressure Gastroenterology Varicose Veins 03 medical and health sciences 0302 clinical medicine Interquartile range Internal medicine Hypertension Portal medicine Humans Decompensation Hepatology medicine.diagnostic_test Vascular disease business.industry medicine.disease Portal vein thrombosis 030220 oncology & carcinogenesis Liver biopsy Portal hypertension 030211 gastroenterology & hepatology business |
| Zdroj: | Clinical Gastroenterology and Hepatology. 20:e251-e266 |
| ISSN: | 1542-3565 |
| DOI: | 10.1016/j.cgh.2020.11.039 |
| Popis: | Background & Aims Porto-sinusoidal vascular disease (PSVD) was recently proposed as novel clinical entity characterized by typical histological changes with or without portal hypertension (PH) in the absence of cirrhosis. Thus, we aimed to describe clinical characteristics and the outcome of PSVD patients and to compare these to patients meeting traditional idiopathic non-cirrhotic portal hypertension (INCPH) criteria. Methods Patients undergoing liver biopsy (baseline) ±hepatic venous pressure gradient (HVPG) measurement at the Vienna General Hospital between 2000-2019 were screened for PSVD and INCPH criteria. Results 91 patients were diagnosed with PSVD of which 28 (30.8%) also fulfilled INCPH criteria (INCPH+/PSVD+). Specific histological and specific clinical PH signs were found in 72 (79.1%) and 54 (59.3%) patients, respectively. INCPH+/PSVD+ showed higher Child-Pugh-scores (7±2 vs 6±1 points; P = .002) and a higher prevalence of decompensation (57.1% vs 28.6%; P = .009) than INCPH-/PSVD+ patients. Importantly, hepatic decompensation after three years (3Y) occurred in 11.2% of PSVD patients with specific clinical signs of PH, while no decompensation occurred in patients with only specific histological or with unspecific clinical/histological signs (P = .002). When categorizing by INCPH definition, 3Y decompensation was 13.4% in INCPH+/PSVD+ and 3.8% in INCPH-/PSVD+ (P = .120). While overall mortality was similar in INCPH+/PSVD+ (n = 6; 21.4%) and INCPH-/PSVD+ (n = 10; 15.9%) patients (P = .558), liver-related mortality tended to be higher in INCPH+/PSVD+ (6.9%) than in INCPH-/PSVD+ (0%; P = .078). Conclusion Novel PSVD criteria facilitate diagnosis. Compared to INCPH, clinical course of PSVD patients is more favorable. Importantly, specific signs of PH including varices and collaterals are associated with hepatic decompensation and mortality. |
| Databáze: | OpenAIRE |
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