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Common Variable Immunodeficiency
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DOI：10.1016/j.mcna.2023.06.012.png)
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Abstract
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CVID is characterized as a heterogenous group of disorders exhibiting a common phenotype of abnormal B-cell differentiation with impaired production of specific immunoglobulin. The clinical manifestations are variable and include acquisition of recurrent bacterial infections, lymphoproliferation, autoimmunity, pulmonary disease, gastrointestinal disease, granulomas, and an increased risk of cancer. Although many different types of infections can occur, sinopulmonary infections are the most common. The onset of the disease can be at any age, but the peak incidence occurs in ages 16 to 20 years. The diagnosis of CVID is complex, as there is not one specific laboratory finding associated with the disease. There are several clinical criteria models used in practice, all of which indicate patients should have hypogammaglobulinemia, defective vaccine responses, age greater than 4 years, and exclusion of other causes of immunoglobulin deficiency. The management of CVID consists of regular immunoglobulin replacement therapy, prevention of infections, and vigilant surveillance for complications.
Keywords:
Common variable immunodeficiency
Primary immune deficiency
Autoimmunity
Hypogammaglobulinemia
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Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency
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