CVID (Common variable immunodeficiency) is the most common subtype of primary immunodeficiencies. At ICID in Aarhus University Hospital we have approximately 50 CVID patients, which makes it our largest patient group.

CVID is defined as congenital lack of antibodies. The symptoms first are seen among young adults as repeated episodes of respiratory infections such as pneumonia and sinusitis. The multiple and long lasting infections can lead to damage of the lungs and lower the lung function.

CVID is not only an immunodeficiency. Every fifth CVID patient suffers from autoimmunity to some extent. This means that the body either overreacts to an infection or it reacts against itself. These patients have an uncontrolled immune system that will cause undesirable reactions in the body.

The aim of the project

Through this research project we want to get a better understanding of the underlying mechanism of CVID. Fore example we want to understand why the patients are very sensitive to a certain spectrum of microorganisms. We only know very little about CVID, so the first goal is to obtain knowledge about the basal immunology, which can be used to develop better treatment methods in the longer terms.

We also try to understand why CIVD expresses it self in very different ways among the patients. Some of them suddenly get a low platelet count or anemia, which increases the risk of getting more serious diseases. We want to investigate if it’s possible to predict who has the biggest risk of developing more serious diseases such as autoimmunity, blood cancer, lymph cancer and cancer in the ventricle among the CVID patients. If we were able to identify the group of risk it we would be able to be more observant around these patients and try to prevent complications.

The possible association with regulatory B-cells

It is known that among other autoimmune diseases such as sclerosis and diabetes the regulatory B-cells are an important factor in the development of the disease.
The regulatory B-cells are a subtype of B-cells that are used in the control of inflammation due to an infection. They also prevent the immune system from reacting against the tissues of the body itself. Our theory is that the regulatory B-cells are involved in the development of the autoimmunity that is seen among some CVID patients. Therefore we want to investigate if there is a association between abnormal counts and function of regulatory B-cells and the autoimmunity. If this turns out to be the case, we will be able to identify the group of risk and thereby be able to offer better and more specific treatment.