Lymphocyte Immunophenotyping in Common Variable Immunodeficiency

NCT ID: NCT01196702

Last Updated: 2024-03-26

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 210 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2020-09-01
• Study Completion Date: 2023-12-30

Brief Summary

The purpose of this study is to discover if differences in the surface markers of B-cells (antibody producing cells of the immune system) in Common Variable Immune Deficiency (CVID) are related to CVID or its complications/treatment (e.g. bronchiectasis, granulomatous disease, immunoglobulin treatment).

The study hypothesis is that the altered B-cell surface markers are related to CVID, and not to the complications or treatment of CVID.

Detailed Description

Common Variable Immune Deficiency (CVID) is a syndrome containing a spectrum of disorders which results in weakened immunity and recurrent infections. The ESID (European Society for Immunodeficiencies) CVID definition includes patients with marked decrease of IgG (at least 2 standard deviations below the mean for age). Patients must also have disease onset at an age over 2 years, absent isohaemagglutinins and/or response to vaccines and other defined causes of hypogammaglobulinaemia must be excluded. The Euroclass system of classifying CVID is the result of a European multicentre trial attempting to develop a consensus of two existing classification schemes of B-cell immunophenotyping. In this paper it was shown that B-cell immunophenotype correlated with coincidence of clinical sequelae and it suggested implementing this to further classify CVID to give prognostic and therapeutic information. However, it has not yet been shown that these alterations in B-cell immunophenotype are the result of CVID itself and not caused by the treatment or complications of CVID (e.g. immunoglobulin replacement therapy, granulomatous disease, bronchiectasis). The aim of this study is to show that alterations in B-cell immunophenotype are caused by CVID itself and not by its complications or treatment. The study will therefore compare CVID patients to suitable control patients with granulomatous disease, bronchiectasis and on long-term immunoglobulin therapy. A control group of normal people will also be included to ensure the assay can detect normality and to show differences between normal people and patients with CVID.

Conditions

Common Variable Immunodeficiency; Granulomatous Disease; Bronchiectasis; Immunoglobulin Treatment

Study Design

• Observational Model Type: CASE_CONTROL
• Study Time Perspective: CROSS_SECTIONAL

Study Groups

CVID

Patients with common variable immunodeficiency

No interventions assigned to this group

CVID and granulomatous disease

Patients with CVID complicated with granulomatous inflammation

No interventions assigned to this group

CVID and bronchiectasis

Patients with CVID complicated by bronchiectasis

No interventions assigned to this group

Control on Immunoglobulin

Patients on immunoglobulin long-term who do not have an immunodeficiency

No interventions assigned to this group

Control bronchiectasis

Controls with bronchiectasis not caused by a known immunodeficiency

No interventions assigned to this group

Control with granulomatous disease

Control patients with Crohn's Disease as this is a disease that causes granulomatous inflammation.

No interventions assigned to this group

Healthy Controls

No interventions assigned to this group

Eligibility Criteria

Inclusion Criteria

• 18 or over
• Competent to consent
• Have diagnosis of Common Variable Immunodeficiency, granulomatous disease, on long term immunoglobulin or bronchiectasis.

Exclusion Criteria

• Under 18
• Unable to consent.

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Barts & The London NHS Trust

OTHER

Sponsor Role: lead

Responsible Party

Mathew Buckland

Consultant Immunologist

Responsibility Role: PRINCIPAL_INVESTIGATOR

Locations

Barts and the London NHS Trust

London, , United Kingdom

Countries

United Kingdom

References

Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, Vlkova M, Hernandez M, Detkova D, Bos PR, Poerksen G, von Bernuth H, Baumann U, Goldacker S, Gutenberger S, Schlesier M, Bergeron-van der Cruyssen F, Le Garff M, Debre P, Jacobs R, Jones J, Bateman E, Litzman J, van Hagen PM, Plebani A, Schmidt RE, Thon V, Quinti I, Espanol T, Webster AD, Chapel H, Vihinen M, Oksenhendler E, Peter HH, Warnatz K. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2008 Jan 1;111(1):77-85. doi: 10.1182/blood-2007-06-091744. Epub 2007 Sep 26.

Reference Type: BACKGROUND

PMID: 17898316 (View on PubMed)

Other Identifiers

006749

Identifier Type: -

Identifier Source: org_study_id