eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

IgA and IgG Subclass Deficiencies: Follow-up

Author: Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Contributor Information and Disclosures

Updated: Feb 19, 2009

Follow-up

Further Outpatient Care

  • Because immunoglobulin (Ig)G subclass and/or isolated IgA deficiency are observed most commonly in atopic or autoimmune disorders, treating the concomitant diseases is crucial. Adequate treatment for the underlying allergic component may substantially decrease the rate of chronic upper and lower respiratory infections.
  • Live viral vaccines are not a risk in partial defects. When the diagnosis of partial defects is established, live viral vaccines can be administered. However, the risk occurs when the exact diagnosis is not known. Until concomitant T-cell deficiency is ruled out, live viral vaccines should be avoided in the patient and in siblings or other children in the house because the attenuated virus is excreted and poses a threat to the severely immunodeficient patient.
  • Sorensen et al (1998) showed that a significant percentage of children with specific antibody deficiency develop protective antibody levels to the conjugated pneumococcal vaccine (Prevnar) with subsequent decreased infections.37
  • In addition, serum IgA subclass levels, IgG subclass levels, or both may increase to normal range in more than 50%.29 Therefore, regular monitoring of immunoglobulin levels should be done. Conversely, levels may decrease and lose specific immunoglobulin production as some patients progress to common variable immunodeficiency (CVID).
  • Do not make a decision to start IVIg treatment lightly. Although its beneficial effect for patients with agammaglobulinemia or hypogammaglobulinemia is not controversial, its use in IgG subclass deficiency or specific antibody deficiency in the presence of normal total Igs is not routine. In 1999, Lawton pointed out that patients, their parents, or the referring physician often insist on a trial of Ig therapy.38 When this results in a beneficial effect, discontinuation is often difficult because "the child's health and family peace soon become dependent on continuation of the treatment."38
  • In addition to the high cost of IVIg and the emotional stress of monthly infusions, keep in mind the real possibility of IVIg transmitting unknown infectious agents. Hepatitis C virus transmission has been documented with certain IVIg preparations.
  • Families usually agree to a predetermined interval for an empiric trial of IVIg if the clinical problems with recurrent upper and lower respiratory infections appear to be sufficiently severe in patients younger than 4 years. This may occur during the winter, ending in spring. Immunologic testing then can be repeated 3-4 months after the last infusion. Restart treatment only if the antibody response remains abnormal.

Complications

  • Prevent chronic upper and lower respiratory disorders by treating underlying allergic disorders (eg, rhinitis, asthma) with aggressive use of antibiotics to manage bacterial infections (eg, sinusitis, bronchitis, pneumonias). Chronic ear infections can cause hearing loss. Watch for mastoiditis.
  • In one study, chronic pulmonary damage, as determined on chest CT scanning, occurred in about 20% of patients.28 In the converse, extensive evaluation of patients with bronchiectasis showed that 11% had polysaccharide antibody deficiency to either pneumococcal or HIB vaccines.15
  • No good studies have been conducted to examine the potential benefits of prophylactic antibiotics, given systemically or topically (ie, aerosolized), in patients with B-cell disorder. However, it can be invaluable in selected patients.
  • Patients with impaired responses to vaccines may eventually develop CVID, possibly with concomitant T-cell deficiency. Closely follow up such patients and serially assess specific antibody responses (protein and polysaccharide antigens) and IgG subclasses and/or IgA serum levels.
  • Some IgA deficiency also occurs in patients with T lymphocyte deficiency such as ataxia-telangiectasia. Live viral vaccines should be avoided in these patients.

Prognosis

  • The prognosis of patients with these B-cell disorders depends on the severity of the humoral deficiency and on the extent to which other associated disorders, such as atopic or autoimmune diseases, are involved.
  • IVIg therapy offers a good prognosis for patients with the most severe form of antibody deficiency (ie, agammaglobulinemia).
  • In many individuals, selective IgA deficiency occurs without respiratory or intestinal symptoms. The role of selective IgG deficiency role in susceptibility to infections is similarly uncertain because many individuals with selective IgG deficiency are asymptomatic.
  • Most patients with IgA and IgG subclass deficiency who are younger than 4 years do well with appropriate antibiotic therapy and outgrow their frequent or recurrent infections, especially if they can make specific antibodies. Closely monitor patients who have IgG subclass deficiency that does not improve to assess for relatively complete B-cell deficiency such as CVID.8
  • Wolpert and Knutsen reported on 120 children with specific antibody deficiency and found that 50% developed normal responses after a mean of 3.1 years.39 IVIg was administered in 28% of these children. Similar findings were reported by Kutukculer et al.29

Patient Education

  • Because of the close association with various allergic and autoimmune disorders, parents and patients must be aware of any underlying chronic atopic disorders.
  • Possible allergic triggers may require additional intervention with allergy testing and medical treatment.

Miscellaneous

Medicolegal Pitfalls

  • In monitoring patients with B-cell deficiency, one must first rule out concomitant T-cell deficiency, especially ataxia telangiectasia or common variable immunodeficiency (CVID).
  • Physicians who are uncomfortable interpreting the results of various immune function testing, especially age-dependent levels of Igs and their subclasses, should refer the patient to a specialist in immunology. Most clinical laboratories do not report age-appropriate normal ranges. In addition, reporting of IgG subclass levels widely varies among laboratories.

Special Concerns

 


More on IgA and IgG Subclass Deficiencies

Overview: IgA and IgG Subclass Deficiencies
Differential Diagnoses & Workup: IgA and IgG Subclass Deficiencies
Treatment & Medication: IgA and IgG Subclass Deficiencies
Follow-up: IgA and IgG Subclass Deficiencies
References
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References

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Further Reading

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Keywords

IgG subclass deficiency, B-cell disorders, immunoglobulin A deficiency, IgA deficiency, immunoglobulin M deficiency, IgM deficiency, immunoglobulin G subclass deficiency, IgG subclass deficiency, antigen-specific antibody deficiency, ASAD, specific antibody deficiency, SAD, bronchiectasis, asthma, sinusitis, combined variable immunodeficiency, CVID, panhypogammaglobulinemia, atopic asthma, allergic rhinitis, atopic dermatitis, IgE-mediated food allergy, gastric carcinomas, lymphomas, rheumatoid arthritis, systemic lupus erythematosus, pernicious anemia, immune thrombocytopenic purpura, ITP, celiac, ulcerative colitis, regional enteritis, Streptococcus pneumoniae, Haemophilus influenzae, Branhamella catarrhalis, Staphylococcus aureus, otitis media, otorrhea, facial abnormalities, ear abnormalities, hand abnormalities, growth retardation, muscular hypotonia, mental retardation, Wolf-Hirschhorn syndrome

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Contributor Information and Disclosures

Author

Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Terry Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Ann O'Neill Shigeoka, MD †, Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine
Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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