Patients have a history of recurrent typical infections with normal and IgG subclasses and failure to respond to specific antigens (test immunization). Many patients are clinically normal, some have recurrent bacterial sinopulmonary infections (Haemophilus, Pneumococcus, Moraxella), complicated by chronic lung disease and bronchiectasis. Serum immunoglobulin levels are normal or elevated. There are low specific antibodies, to capsulated organisms, and poor responses to test immunization, and to polysaccharide antigens.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- (Intravenous) immunoglobulins. Antibiotic therapy together with physiotherapy and postural drainage in case of lung damage. Ciprofloxacin has not been licensed for small children. Oral poliovaccine should not be given because there is a risk of paralytic disease.
- Common Variable Immunodeficiency (CVID), eMedicine
Research programs, clinical trials
- European Initiative for Primary Immunodeficiencies
- Improved Healthcare for Patients with Primary Antibody Deficiencies through new Strategies Elucidating their Pathophysiology (IMPAD), IMPAD
- The Genetics of IgA Deficiency and Common Variable Immune Deficiency, Comprehensive Cancer Center, University of Albama at Birmingham
- Immune Regulation in Patients with Common Variable Immunodeficiency and Related Syndromes, ClinicalTrials.gov