PSVD-India: Clinical Diagnosis and Pathological Spectrum of Porto-sinusoidal Vascular Disease in India

Sponsor

Postgraduate Institute of Medical Education and Research (Other)

Overall Status

Recruiting

CT.gov ID

NCT06054451

Collaborator

(none)

210

Enrollment

Location

Anticipated Duration (Months)

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

There is a need to re-evaluate the patients classified as NCPH and determine whether the new histological classification proposed by the VALDIG applies to the Indian scenario. We intend to identify the patient cohorts who have been diagnosed as NCPH, NCPF, EHPVO, hepatic venous outlet tract obstruction (HVOTO), Veno-occlusive disease (VOD) and sinusoidal obstruction syndrome (SOS) based on their liver biopsy, endoscopy, HVPG, and radiology reports. These patients will be screened to find the patients who fit the diagnosis of PSVD. It is important to establish whether the new definition of PSVD is relevant to the Indian population and establish the usefulness of invasive tests like liver biopsy in diagnosing the disease. The patient cohorts meeting diagnosis of INCPH will be compared with those meeting the new diagnosis of PSVD. The investigators will describe the clinical (demographic, clinical risk factors, socioeconomic status), etiological (associated conditions, coagulation disorders medication use, genetic risk factors), imaging (based on ultrasound Doppler imaging or cross- sectional imaging), endoscopic, fibrosis tests (using non-invasive tests), and the histopathology of the patients who fulfil the criteria of PSVD.

Condition or Disease	Intervention/Treatment	Phase
Portal Hypertension Budd-Chiari Syndrome Non-Cirrhotic Portal Hypertension Porto-Sinusoidal Vascular Diseases Sinusoidal Obstruction Syndrome Veno Occlusive Disease, Hepatic Non-Cirrhotic Portal Fibrosis

Detailed Description

Portal hypertension is clinically characterized by a portal venous pressure gradient between the inferior vena cava and the portal vein is more than 5 mm. Liver cirrhosis remains the leading cause of portal hypertension in the western world. However, there are conditions where portal hypertension can even occur without liver cirrhosis, referred to as Noncirrhotic portal hypertension (NCPH). The term NCPH has been derived from a variety of histopathological entities such as hepatoportal sclerosis, noncirrhotic portal fibrosis, nodular regenerative hyperplasia, or incomplete septal fibrosis. In the absence of other causes of cirrhosis and portal venous thrombosis, the condition is termed idiopathic noncirrhotic portal hypertension. The histopathological findings of INCPH are not entirely specific to portal hypertension, similar changes can be seen in patients without portal hypertension. Therefore the term Porto-sinusoidal vascular disorder(PSVD) has been recently introduced to define a group of liver vessel disorders affecting the portal venules and sinusoids irrespective of the absence or presence of liver cirrhosis. This disease entity can have a varied clinical presentation and histological findings and has been associated with several immunological and systemic disorders.

Porto-sinusoidal vascular disease - Currently, for clinical practice, the VALDIG group has given a working definition for PSVD.

"Liver biopsy ≥20mm without cirrhosis with either 1 specific sign of portal hypertension OR 1 specific histological finding for PSVD" OR "Liver biopsy ≥20mm without cirrhosis with 1 sign not specific for portal hypertension AND 1 sign not specific for PSVD"

Study Design

Study Type:

Observational

Anticipated Enrollment :

210 participants

Observational Model:

Case-Only

Time Perspective:

Prospective

Official Title:

Clinical Diagnosis and Pathological Spectrum of Porto-sinusoidal Vascular Disease in India

Actual Study Start Date :

Aug 1, 2023

Anticipated Primary Completion Date :

Jun 1, 2024

Anticipated Study Completion Date :

Jun 1, 2024

Outcome Measures

Primary Outcome Measures

To describe the clinical presentation of patients with a diagnosis of PSVD. [Day 0]
to compare the clinical diagnosis of NCPH of PSVD

Secondary Outcome Measures

To define the radiological description of patients with PSVD [Day 0]
Radiological assessment will be done at baseline for characterization
To define histopathological features and subtypes of the PSVD spectrum in patients from India [Day 0]
To classify the PSVD based on the histological finding the disease.
To describe the findings of non-invasive techniques like transient elastography in diagnosis of PSVD [Day 0]
To look for the trend of non-invasive techniques in PSVD such as ARFI, transient elastography, coagulation parameters, and inflammatory biomarkers in patients of PSVD
To describe the findings of biomarkers in diagnosis of PSVD [Day 0]
To look for the trend of non-invasive techniques in PSVD such as coagulation parameters, and inflammatory biomarkers in patients of PSVD
To describe the endoscopic findings in patients with PSVD [Day 0]
To look for esophageal varices and portal hypertensive gastropathy
To describe the pathological characteristics of patients with a diagnosis of PSVD. [Day 0]