Common variable immune deficiency
From IDWiki
Clinical Manifestations
- Recurrent sinopulmonary infections
- Chronic diarrhea, including IBD-like disease
- Can have concurrent autoimmune disease, including ITP, AIHA, rheumatoid arthritis, pernicious anemia, autoimmune thyroiditis, and vitiligo
- Can have signs and symptoms of allergic rhinitis and asthma
- Can have splenomegaly and lymphadenopathy
- Increased risk of non-Hodgkin lymphoma
Differential Diagnosis
- Primary hypogammaglobulinemia
- IgG subclass deficiency
- Hyperimmunoglobulin M syndrome
- Other combined immunodeficiency
- Secondary hypogammaglobulinemia
- Drugs, including rituximab, CAR-T, BiTE, long-term corticosteroids, certain antiseizure medications, cytotoxic chemotherapy
- Malignancy, including CLL, lymphoma, multiple myeloma, and Waldenström macroglobulinemia
- Thymoma with hypogammaglobulinemia (Good syndrome)
Diagnosis
- Over the age of 4 years
- All of the following:
- Low IgA and/or IgM
- Poor or absent response to immunization
- Absence of other immunodeficiency
