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

NCT06145100 | Completed

• 1 other identifier: CVID-pHT
• Study Type: observational
• Target: 250
• Locations: 1 country
• Sites: 1

Brief 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.

Conditions

Non-Cirrhotic Portal Hypertension; Common Variable Immunodeficiency

Keywords

non-cirrhotic portal hypertension; Common Variable Immunodeficiency; ultrasound; liver stiffness measurement; spleen stiffness measurement

Outcome Measures

Primary Outcomes (2)

• 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

• 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 Outcomes (6)

• Differences in portal vein diameter between CVID patients with and without liver involvement and with clinically significant portal hypertension.

  Study inclusion, after 12 months

• Differences in portal vein flow velocity between CVID patients with and without liver involvement and with clinically significant portal hypertension.

  Study inclusion, after 12 months

• Differences in alanine aminotransfersases (ALT) between CVID patients with and without liver involvement and with clinically significant portal hypertension.

  Study inclusion, after 12 months

• Differences in aspartat aminotransfersases (AST) between CVID patients with and without liver involvement and with clinically significant portal hypertension.

  Study inclusion, after 12 months

• Differences in liver function tests (bilirubin, albumin, INR) between CVID patients with and without liver involvement and with clinically significant portal hypertension.

  Study inclusion, after 12 months

Study Arms (3)

Patients with CVID and autoimmunity and no liver involvement.

Patients with CVID and autoimmune disease, but no liver involvement (normal liver enzymes and normal abdominal ultrasound)

Diagnostic Test: Ultrasound including color doppler ultrasound

Patients with CVID and liver involvement and no clinical significant portal hypertension

Patients with CVID and liver involvement (elevated liver enzymes, abnormal ultrasound and/or abnormal liver biopsy), but no clinically significant portal hypertension

Diagnostic Test: Ultrasound including color doppler ultrasound

Patients with CVID and clinically significant non-cirrhotic portal hypertension

Patients with CVID and clinically significant non-cirrhotic portal hypertension (indicated by the presence of ascites or esophageal varices / portal-hypertensive gastropathy).

Diagnostic Test: Ultrasound including color doppler ultrasound

Interventions

Ultrasound including color doppler ultrasound DIAGNOSTIC_TEST

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.

Also known as: Liver stiffness measurement by transient elastography, Spleen stiffness measurement by transient elastography

Patients with CVID and autoimmunity and no liver involvement.; Patients with CVID and clinically significant non-cirrhotic portal hypertension; Patients with CVID and liver involvement and no clinical significant portal hypertension

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with CVID will be included in the study. Patients will be divided into three subgroups: 1) Patients with CVID and autoimmunity, but no liver involvement (n=75) 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 (n=75) 3) Patients with CVID and clinically significant non-cirrhotic portal hypertension (n=75). 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 (n=25)

You may qualify if:

• Patients with CVID

You may not qualify if:

• 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)

Sponsors & Collaborators

• University Hospital Freiburg: lead

Study Sites (1)

University Medical Center Freiburg, Department of Medicine II

Freiburg im Breisgau, 79106, Germany

Location

Biospecimen

Retention: SAMPLES WITHOUT DNA

Serum, plasma, peripheral blood mononuclear cell (PBMC)

MeSH Terms

Conditions

Common Variable Immunodeficiency

Condition Hierarchy (Ancestors)

Immunologic Deficiency Syndromes; Immune System Diseases

Study Officials

• Dominik Bettinger, MD

  University Hospital Freiburg

  PRINCIPAL INVESTIGATOR

• Klaus Warnatz, MD

  University Hospital Freiburg

  PRINCIPAL INVESTIGATOR

• Marlene Reincke, MD

  University Hospital Freiburg

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: November 10, 2023
• First Posted: November 22, 2023
• Study Start: November 1, 2023
• Primary Completion: December 31, 2025
• Study Completion: March 30, 2026
• Last Updated: May 1, 2026

Record last verified: 2026-04

Locations

DE (1)