Characterization of porto-sinusoidal vascular disease in patients with common variable immunodeficiency.

Apr 11, 2024

Cross-sectional observational study of a multicenter cohort of patients with common variable immunodeficiency.
– Main variable: porto-sinusoidal vascular disease. With or without portal hypertension.
Secondary variables: demographic, clinical, analytical, hemodynamic, radiological, and histological data where available, as well as the patient’s clinical evolution.

– Study phases:
1) Patient identification and data collection: Identification of patients in each center and collection of demographic, clinical, and analytical data. Hemodynamic, radiological and anatomopathological (in the subgroup of patients with signs of portal hypertension) and quality of life tests will also be conducted. Endoscopic studies (according to the physician’s criteria) will be recorded.
2) Portal hypertension data collection: Hepatic and splenic transition elastography (where available), abdominal Doppler ultrasound, basic hepatopathy blood tests (liver function, coagulopathy, etiology). In each center, endoscopic studies (gastroscopy +/- colonoscopy) performed according to physician criteria will be registered. As part of routine clinical practice, portal hypertension study will be completed with hepatic CT/MRI, portal hemodynamics (if available), and liver biopsy only in patients showing suggestive alterations such as changes on abdominal ultrasound (splenomegaly, structural alteration of hepatic parenchyma, vena umbilical repermeabilization, appearance of shunts, presence of porto-systemic collateral circulation, enlarged portal vein, etc.), fibrosis criteria by elastography, or analytical alterations (elevated transaminases or bilirubin, thrombocytopenia, or prolonged INR) once informed consent is obtained.
3) In the cohort of patients with CVID from each hospital: Data specified in section 2 will be collected, along with any complications or decompensations (as per Baveno VII: ascites, hepatic encephalopathy, variceal hemorrhage) presented up to the review date.


Project recruiting


Primary Objective:

To identify and characterize the presence of Porto-Sinusoidal Vascular Disease in patients with common variable immunodeficiency.


Secondary Objectives:

  1. Compare clinical, laboratory, radiological, and hemodynamic features in patients with common variable immunodeficiency with and without PSVD.
  2. Evaluate and compare the progression of PSVD with/without portal hypertension in patients with common variable immunodeficiency.
  3. Assess the impact of the presence of PSVD and portal hypertension in patients with CVID on mortality.
  4. Identify surrogate markers of clinical decompensation in patients with PSVD through collected data (clinical, laboratory, radiological, and hemodynamic).
  5. Evaluate the quality of life perception in patients with common variable immunodeficiency with and without PSVD.

Study file(s)


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