Sinus infections, ear infections, pneumonia, and other upper respiratory infections that are severe, frequent, or long-lasting are considered recurrent infections. Recurrent infections may be a sign of immunodeficiency requiring treatment by an allergist or immunologist. Specific antibody deficiency (SAD) and Common variable immune deficiency (CVID) are two disorders that Dr. Beerelli treats.
Overview of Specific Antibody Deficiency
Specific antibody deficiency (SAD) is a condition where individuals do not produce an adequate antibody response to polysaccharide antigens which are often encapsulated around the surface of bacteria. As a result, individuals with SAD have recurrent respiratory infections that are not well controlled with antibiotics. SAD is a frequent diagnosis for adults and children older than 2 years who experience recurrent rhinosinusitis and/or bronchopulmonary infections but have otherwise normal responses to protein antigens and normal serum levels of immunoglobulins. SAD usually resolves spontaneously in young children over time. However, in older children and adults, SAD can persist with mild to moderate severity and may require management with appropriate therapy.
Overview of Common Variable Immunodeficiency
CVID is a disorder where the immune system’s B cells are impaired and do not produce enough immunoglobulin, resulting in hypogammaglobulinemia. Because the immune system is defective, patients with CVID often experience recurrent infection. CVID affects both children and adults and it is “variable” because of the many different clinical presentations of the disorder and the fact that it is not just one disease, but a group of diseases resulting from genetic mutation. CVID can be diagnosed in childhood, although it is typically discovered after puberty with most diagnoses occurring between 20 and 40 years of age.
Symptoms of Recurrent Infections
Symptoms of Specific Antibody Deficiency
The primary symptoms of specific antibody deficiency are recurrent ear, sinus, and/or lung infections.
Recurrent infections are:
- 2 or more severe infections in 1 year that involve persistent fever, poor response to oral antibiotics, and hospitalization/ bed confinement for 1 or more weeks.
- 3 or more respiratory (sinusitis, otitis, bronchitis) infections in 1 year.
- Infections that require antibiotics for 2 months/year.
Patients may also present with atopic dermatitis (eczema rash) or asthma.
Symptoms of Common Variable Immunodeficiency
Most patients with common variable immunodeficiency (CVID) also experience recurrent infections. In fact, only a small percentage of CVID patients (8-10%) have little or no medical history of infection. In addition to recurrent infections, CVID patients may display symptoms related to other conditions. These include:
- Autoimmune disease
- Hematologic disorders
- Severe allergies
- Chronic lung disease
- Gastrointestinal disorders
- Liver disorders
A detailed history and physical exam is conducted. After obtaining a detailed history of your previous infections, symptoms, responses to immunizations and antibiotics, as well as family immunologic history, your allergist/immunologist may order additional laboratory studies to evaluate for specific antibody deficiency (SAD) or Common Variable Immunodeficiency (CVID).
Specific tests to evaluate for SAD or CVID may include:
- Serum level measurements of IgG, IgA, and IgM
- IgG subclass level measurements
- Performing a vaccine challenge to assess your immune response to polysaccharide versus protein vaccines
Treatment of Recurrent Infections
All patients with specific antibody deficiency (SAD) should be immunized with the 13-valent pneumococcal conjugate vaccine or PCV-13 vaccine. Before initiating any therapy for Common Variable Immunodeficiency (CVID) or SAD, patients who have active infection should have their infection treated first. Antibiotics may need to be administered.
Treatment & Management of Specific Antibody Deficiency
For mild to moderate cases of SAD, aggressive treatment of other existing conditions, such as asthma and allergic rhinitis, can be initiated. In addition, prophylactic antibiotics can be prescribed. Severe cases of SAD may require immunoglobulin replacement. Often, young children can outgrow SAD and discontinue replacement therapy. However, older children and adults may have persistent disease requiring continuous replacement therapy.
Treatment & Management of Common Variable Immunodeficiency
Immune globulin replacement may slow the progression of chronic lung disease and provide some protection against autoimmune disorders, although this is not well studied. Your allergist/immunologist may decide to postpone immune globulin replacement therapy if you have higher levels of serum IgG with only minor impaired response to vaccines. However, you should be continuously monitored every 6 to 12 months.
The safety and efficacy of low dose Interleukin-2 (IL-2) treatment is still under investigation. A small number of allogeneic stem cell transplants have been performed in CVID patients.
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