Porto-sinusoidal vascular disorder.

Area of Science:

• Hepatology
• Vascular Biology
• Liver Pathology

Background:

• Portal hypertension can exist without cirrhosis, linked to immune disorders, infections, and thrombophilia.
• Hepatic sinusoidal and (peri)portal vascular abnormalities occur even without portal hypertension.
• Porto-sinusoidal vascular disorder (PSVD) is a recently introduced term for liver vascular diseases affecting portal venules and sinusoids, regardless of portal hypertension.

Purpose of the Study:

• To define Porto-sinusoidal Vascular Disorder (PSVD) as a distinct clinical entity.
• To highlight diagnostic criteria and potential co-existing liver conditions in PSVD.
• To emphasize the importance of PSVD in facilitating future research.

Main Methods:

• Liver biopsy is fundamental for diagnosing PSVD.
• Histological findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis, and incomplete septal fibrosis/cirrhosis.
• Clinical criteria, imaging, and non-invasive tests like liver and spleen stiffness measurements aid diagnosis.

Main Results:

• PSVD encompasses a spectrum of liver vascular lesions.
• Differential diagnosis must consider co-existing conditions like fatty liver disease, viral hepatitis, or portal vein thrombosis.
• Histology is key, supported by imaging and non-invasive tests.

Conclusions:

• The establishment of PSVD as a novel clinical entity will foster collaborative research.
• Understanding PSVD's molecular pathomechanisms is a future research direction.
• Accurate diagnosis and assessment of contributing factors are crucial for patient management.