Prediction of Portal Hypertension in Patients With CVID (CVID-pHT)

Common Variable Immunodeficiency Clinical Trial

— CVID-pHT  

Official title:

Evaluation of Ultrasound Parameters and Liver and Spleen Stiffness for Prediction of Clinically Significant Portal Hypertension in Patients With Common Variable Immunodeficiency Syndrome

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT06145100
• Other study ID #: CVID-pHT
• Status: Recruiting
• Start date: November 1, 2023
• Est. completion date: December 31, 2025

Study information

• Verified date: November 2023
• Source: University Hospital Freiburg
• Contact: Dominik Bettinger, MD
• Phone: +49761270-36870
• Email: dominik.bettinger[@]uniklinik-freiburg.de
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

Patients with CVID will be offered to participate in this observational trial during the routine annual visit in the outpatient clinic at the Center of chronic Immunodeficiency (CCI) of the University Medical Center Freiburg, Germany.

Clinical and laboratory data at the time of presentation will be assessed. Additionally, parameters of abdominal ultrasound, duplex sonography of the liver and spleen, and liver and spleen stiffness at the time of presentation will be evaluated. If applicable, clinical and/or interventional parameters indicating clinically significant portal hypertension (i.e. presence of varices or portal-hypertensive gastropathy in esophago-gastroduodenoscopy, presence of ascites) within 12 months prior and after the index visit will be assessed. During the visit, serum/plasma samples and peripheral blood mononuclear cells (PBMC) are collected and stored in an associated biobank.

Description:

Patients with CVID can be included in the study. The study cohort will consist of three subgroups: 1) Patients with CVID and autoimmunity, but no liver involvement. 2) Patients with CVID and liver involvement, defined by elevated liver enzymes, abnormal ultrasound and/or abnormal liver biopsy, but no signs of clinically significant portal hypertension and 3) Patients with CVID and clinically significant non-cirrhotic portal hypertension. Further, patients with CVID and diagnosed non-cirrhotic portal hypertension between 01/01/2004 and the start of the prospective part of this study (01/01/2023) will be included retrospectively in this study.

Patients will be recruited during the routine annual visit in the outpatient clinic at the Center of chronic Immunodeficiency (CCI) of the University Medical Center Freiburg, Germany. During the visit a standard ultrasound protocol, including size and morphology of liver and spleen, duplex-sonography of the liver, and measurement of liver and spleen stiffness via transient elastography will be performed as a part of the routine clinical diagnostic. Results from endoscopy or other interventions (esophago-gastroduodenoscopy, invasive measurement of hepatic venous-portal pressure gradient, or TIPS implantation, if applicable) within 12 months prior and after the index visit will be assessed. All non-invasive and invasive interventions will be conducted solely based on clinical decisions made independently from the study inclusion as the study is designed as an observational study.

Detailed patient characteristics, epidemiologic, clinical, imaging and laboratory parameters will be assessed and included in an electronic database.

Apart from these data, patients will be asked to participate in biobank sampling including serum/plasma and peripheral blood mononuclear cells (PBMC) samples from the peripheral veins.

All patients recruited in this study will be followed-up for at least 12 months.

Outcome parameters include parameters from abdominal ultrasound, duplex-sonography and stiffness of liver and spleen, presence or development of clinically significant portal hypertension and non-invasive fibrosis scores based on laboratory and ultrasound parameters.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 250
• Est. completion date: December 31, 2025
• Est. primary completion date: December 31, 2024
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• Patients with CVID

Exclusion Criteria:

• no written informed consent

• concomitant chronic liver disease (viral hepatitis, alcoholic liver disease, steatitic liver disease, hemochromatosis, primary biliary cholangitis, primary sclerosis cholangitis, M. Wilson, alpha-1-antitrypsin deficiency)

Related Conditions & MeSH terms

• Common Variable Immunodeficiency
• Hypertension
• Hypertension, Portal
• Immunologic Deficiency Syndromes
• Non-Cirrhotic Portal Hypertension

Intervention

Diagnostic Test:
• Ultrasound including color doppler ultrasound
Patients with CVID receive color doppler ultrasound of the liver including assessment of portal vein diameter, portal vein flow velocity, hepatic vein diameter and assessment of arterial perfusion. Further liver and spleen stiffness measurement by transient elastography is performed.

Locations

Country	Name	City	State
Germany	University Medical Center Freiburg, Department of Medicine II	Freiburg

Sponsors (1)

Lead Sponsor	Collaborator
University Hospital Freiburg

Country where clinical trial is conducted

Germany, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Differences in liver stiffness between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Liver stiffness will be assessed by transient elastography.	Study inclusion, after 12 months
Primary	Differences in spleen stiffness between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Spleen stiffness will be assessed by transient elastography.	Study inclusion, after 12 months
Secondary	Differences in portal vein diameter between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Portal vein diameter will be assessed by color doppler ultrasound	Study inclusion, after 12 months
Secondary	Differences in portal vein flow velocity between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Portal vein flow velocity will be assessed by color doppler ultrasound	Study inclusion, after 12 months
Secondary	Differences in alanine aminotransfersases (ALT) between CVID patients with and without liver involvement and with clinically significant portal hypertension.	ALT is assessed during routine clinical practice	Study inclusion, after 12 months
Secondary	Differences in aspartat aminotransfersases (AST) between CVID patients with and without liver involvement and with clinically significant portal hypertension.	AST is assessed during routine clinical practice	Study inclusion, after 12 months
Secondary	Differences in liver function tests (bilirubin, albumin, INR) between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Liver function liver function tests (bilirubin, albumin, INR) are assessed during routine clinical practice	Study inclusion, after 12 months
Secondary	Differences in platelets between CVID patients with and without liver involvement and with clinically significant portal hypertension.	Platelets are assessed during routine clinical practice	Study inclusion, after 12 months