Printable factfile
Patients with CVID present recurrent bacterial infection, autoimmune disease like thrombocytopenia, haemolytic anaemia and organ specific autoimmunity. Nodular lymphoid hyperplasia of bowel is unique to CVID. In severe form of CVID, granulomatous disease with lymphadenopathy is common. The major complications of CVID are due to delay in diagnosis. Patients may have bronchiectasis, chronic sinusitis, unusual infections, such as Campylobacter cholangitis, and Mycoplasma/Ureaplasma arthritis. There is an increase risk of lymphoma and gastric carcinoma. Thymomas (benign or malignant) can also occur and often give rise to myasthenia gravis and haematological problems.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- (Intravenous) immunoglobulins and antibiotic therapy together with physiotherapy and postural drainage in case of lung damage. Oral poliovaccine should not be given because there is risk of paralytic disease. The granulomatous disease responds well to steroids. Splenectomy may be necessary for hyperplenism.
- Common Variable Immunodeficiency (CVID), eMedicine
- Hypogammaglobulinemia, eMedicine
Research programs, clinical trials
- European Initiative for Primary Immunodeficiencies
- Immune System and Gut Abnormalities in Patients with Common Variable Immunodeficiency with and without Gastrointestinal Symptoms, ClinicalTrials.gov
- 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 Cente
- Immune Regulation in Patients with Common Variable Immunodeficiency and Related Syndromes, ClinicalTrials.gov