From the Guidelines
Management of very low Immunoglobulin A (IgA) levels, known as IgA deficiency, primarily focuses on treating infections and addressing complications rather than replacing IgA itself. Most patients with IgA deficiency require no specific treatment as they remain asymptomatic. For those experiencing recurrent infections, prompt antibiotic therapy for bacterial infections is recommended, with consideration of prophylactic antibiotics for patients with frequent infections.
Key Considerations
- Immunoglobulin replacement therapy with intravenous or subcutaneous immunoglobulin (IVIG/SCIG) is generally not indicated for isolated IgA deficiency since commercial preparations contain minimal IgA and don't effectively raise IgA levels 1.
- However, IVIG may be considered in cases with concurrent IgG subclass deficiencies or specific antibody deficiencies.
- Patients should avoid blood products containing IgA if they have anti-IgA antibodies due to risk of anaphylactic reactions.
- Regular monitoring for associated conditions like autoimmune disorders, allergies, and gastrointestinal issues is important, as some patients with IgA deficiency may develop these conditions over time 1.
- Patient education about increased infection risk and prompt treatment of infections is essential.
Diagnosis and Monitoring
- A diagnosis of Selective IgA Deficiency (SIGAD) should be considered in patients older than 4 years with a serum IgA level of less than 7 mg/dL and normal serum IgG and IgM levels, after excluding other causes of hypogammaglobulinemia 1.
- Patients with SIGAD should be monitored over time for the occurrence of complications, including respiratory and gastrointestinal tract infections, atopy, autoimmune diseases, celiac disease, and malignancy 1.
Treatment Approach
- The management approach is individualized because IgA deficiency severity varies widely, with many patients living normal lives without intervention while others require more aggressive infection prevention strategies.
- Immunoglobulin supplementation at replacement doses may be considered for patients with hypogammaglobulinemia and recurrent severe infections, or impaired vaccine responses, in collaboration with an allergist/immunologist 1.
From the Research
Managing Very Low Immunoglobulin A (IgA) Levels
- Very low IgA levels, also known as IgA deficiency, is the most common primary immunoglobulin deficiency, with a prevalence of around one in 500 in Caucasians 2.
- Most individuals with IgA deficiency are asymptomatic, but some may experience recurrent infections, allergic disorders, and autoimmune manifestations 2, 3.
- There is no specific treatment for patients with symptomatic IgA deficiency, but antibiotics may be prescribed for acute infections 2.
- Individuals with IgA deficiency may have anti-IgA antibodies in their serum, which can lead to severe transfusion reactions if they receive blood or blood products containing IgA 2, 4.
Recommendations for Managing IgA Deficiency
- Patients with IgA deficiency and anti-IgA antibodies can receive IgA-depleted intravenous immunoglobulin, which has been shown to be safe and well-tolerated in long-term use 4, 5.
- Prophylactic infusions of intravenous immunoglobulin can help prevent infections in patients with antibody deficiency syndromes, including those with IgA deficiency 5.
- Patients with IgA deficiency should be monitored for associated immune defects, such as IgG subclass deficiency and defective specific antibody production, as these can increase the risk of recurrent infections and bronchiectasis 6.
- Subclassification of IgA deficiency regarding the existence of associated immune defects can be useful in terms of morbidity and planning for medical care 6.