Common variable immune deficiency (CVID) is characterized by humoral immune deficiency with onset after 24 months of age and usually in young adulthood, resulting in increased susceptibility to infections and diminished responses to protein and polysaccharide vaccines. The most common infections are sinopulmonary and include Streptococcus pneumonia, Hemophilis influenza, Klebsiella pneumonia, and sometimes mycoplasma infections. Individuals may experience meningitis or other systemic bacterial infections, recurrent eye or skin infections, or gastrointestinal symptoms related to compromised immune/gut homeostasis, including chronic diarrhea, malabsorption, or bloating. They may also have abnormal T-cell function and immune dysregulation, including lymphoid hyperplasia, gastrointestinal inflammation, autoimmune phenomena, and susceptibility to malignancy, especially lymphomas.
Treatment for CVID includes starting immune globulin replacement therapy to provide protective antibodies as soon as possible, appropriate treatment with antibiotics, and treatment as appropriate for autoimmune phenomena. Administration of purified immune globulin is important in preventing the recurrent sinopulmonary infections associated with CVID. This can now be done via two routes: intravenously (i.e., IVIG) and subcutaneously. Generally, IVIG is dosed at 400 mg/kg every three to four weeks.
Subcutaneous administration of immunoglobulin can be done with a variety of dosing schedules to suit the preference of a patient, with the overall goal of administering a total of 400 mg/kg every three to four weeks. Prophylactic antibiotics may help prevent the recurrent sinopulmonary infections associated with CVID. The goal is to keep IgG levels above 500.
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