Selective IgA deficiency: Management and prognosis
- Robert W Hostoffer, DO, FACOP, FAAP, FACOI, FCCP
Robert W Hostoffer, DO, FACOP, FAAP, FACOI, FCCP
- Associate Professor
- Case Western Reserve University
- Ohio University
INTRODUCTION AND DEFINITION
Selective IgA deficiency (sIgAD) (MIM 137100) is defined as the isolated deficiency of serum immunoglobulin A (IgA) (ie, serum levels of immunoglobulin G [IgG] and immunoglobulin M [IgM] are normal) in a patient older than four years of age in whom other causes of hypogammaglobulinemia have been excluded. It is the most common primary antibody defect in humans.
●Severe IgA deficiency refers to serum levels below 7 mg/dL, which is the lower limit of detection for most assays.
●Partial IgA deficiency refers to serum levels above 7 mg/dL, but below the lower limit of normal (ie, lower than 2 standard deviations below the age-adjusted mean).
The clinical manifestations of sIgAD range from an asymptomatic laboratory finding to recurrent infections and autoimmune disease. This topic reviews the management and prognosis of patients with sIgAD. The clinical features, pathophysiology, and diagnosis are discussed separately. (See "Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis".)
All individuals with selective IgA deficiency (sIgAD) should not only be informed about the associated conditions, but also reassured that the majority of people with this laboratory finding are healthy and live normal lives.
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- Information for patients about selective IgA deficiency is available online and free of charge through the Immune Deficiency Foundation. http://primaryimmune.org/about-primary-immunodeficiency-diseases/types-of-pidd/selective-iga-deficiency (Accessed on April 04, 2013).
- INTRODUCTION AND DEFINITION
- Review medications
- Asymptomatic patients
- - Monitoring
- Symptomatic patients
- - Sinopulmonary infections
- Treat concomitant disorders
- Prophylactic antibiotics
- Immune globulin
- - Giardia infection
- - Autoimmune disorders
- - Reactions to blood products
- Indications for screening for anti-IgA antibodies
- Preparation for future treatment
- Safe administration of blood products
- Risk of malignancy
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS