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

NCT ID: NCT06145100

Last Updated: 2024-12-09

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

250 participants

Study Classification

OBSERVATIONAL

Study Start Date

2023-11-01

Study Completion Date

2025-12-31

Brief Summary

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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.

Detailed Description

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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.

Conditions

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Non-Cirrhotic Portal Hypertension Common Variable Immunodeficiency

Keywords

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

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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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)

Ultrasound including color doppler ultrasound

Intervention Type 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.

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

Ultrasound including color doppler ultrasound

Intervention Type 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.

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).

Ultrasound including color doppler ultrasound

Intervention Type 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.

Interventions

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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.

Intervention Type DIAGNOSTIC_TEST

Other Intervention Names

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Liver stiffness measurement by transient elastography Spleen stiffness measurement by transient elastography

Eligibility Criteria

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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)
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University Hospital Freiburg

OTHER

Sponsor Role lead

Responsible Party

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Prof. Dr. Dominik Bettinger

Principal Investigator

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Dominik Bettinger, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Freiburg

Klaus Warnatz, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Freiburg

Marlene Reincke, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Freiburg

Locations

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University Medical Center Freiburg, Department of Medicine II

Freiburg im Breisgau, , Germany

Site Status RECRUITING

Countries

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Germany

Central Contacts

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Dominik Bettinger, MD

Role: CONTACT

Phone: +49761270-36870

Email: [email protected]

Marlene Reincke, MD

Role: CONTACT

Phone: +49761270-34010

Email: [email protected]

Facility Contacts

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Dominik Bettinger, MD

Role: primary

Marlene Reincke, MD

Role: backup

Other Identifiers

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CVID-pHT

Identifier Type: -

Identifier Source: org_study_id