Bronchiectasis, Common Variable Immunodeficiency, Granulomatous Disease, Immunoglobulin Treatment
Conditions
Keywords
common variable immunodeficiency, granulomatous disease, crohns disease, bronchiectasis, immunoglobulin replacement or treatment, B-cell immunophenotyping
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.
Interventions
None listed
Sponsors
Barts & The London NHS Trust
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
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. * Medical problem that could alter B-cell immunophenotype (except for the diagnoses in the inclusion criteria)/
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Percentage of B-cells of all lymphocytes | 5 months | Look at percentage of cells within the lymphocyte gate that express the B-cell marker CD19, and compare to healthy controls and non-healthy controls. |
| Percentage of class switched memory B-cells as a percentage of B-cells | 5 months | Percentage of class-switched memory B-cells (expressing CD27 and CD19), that do not express IgM or IgD, as a percentage of B-cells. This is reduced in CVID and this will be compared between controls and the patients with CVID. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Percentage expression of CD21 and CD38 | 5 months | Look for abnormalities in the CVID group and compare to control groups in the numeber of B-cells expressing low levels of CD21 (CD21 lo), and high CD38. |
Countries
United Kingdom
Outcome results
None listed