eMedicine Specialties > Allergy and Immunology > Immunodeficiencies

Immunoglobulin A Deficiency: Treatment & Medication

Author: Rebecca Bascom, MD, MPH, Professor of Medicine, Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Pennsylvania State College of Medicine, Milton S Hershey Medical Center; Graduate Faculty Member, Pennsylvania State University College of Medicine and The Huck Institutes of the Life Sciences
Coauthor(s): Marina Y Dolina, MD, Consulting Physician, Lung, Sleep and Critical Care Consultants and Pulmonary Services, Wellspan Health System
Contributor Information and Disclosures

Updated: Sep 29, 2009

Treatment

Medical Care

The approach to treatment includes identification of comorbid conditions; preventive measures to reduce the risk of infection; and prompt, rigorous, and effective treatment of infections. Functional endoscopic sinus surgery can frequently help relieve chronic obstruction and promote drainage. Tympanostomy tubes may also be helpful in reducing the risk of decreased hearing and secondary defective speech development in children with chronic suppurative otitis related to antibody deficiency.

Surgical Care

Some patients with recurrent sinusitis require surgical interventions to promote drainage.

Consultations

  • Rheumatologist
  • Otolaryngologist
  • Allergist/immunologist
  • Pulmonologist
  • Gastroenterologist

Diet

Dietary modifications may be necessary to manage chronic diarrhea and malabsorption or food allergy. A gluten-free diet and, possibly, other restricted diets are important for treatment in patients with celiac disease or who have IgG antibodies to animal proteins in milk or other foods.

Activity

A priori restrictions are not necessary, but patients with chronic lung disease may have decreased exercise tolerance.

Medication

Immunoglobulin A deficiency (IgAD) has no specific treatment. Replacement therapy is not practical for IgAD because of the short half-life of IgA and the relative paucity of IgA in commercial immunoglobulin preparations.

Antibiotic therapy is the first line of treatment, specific for sinopulmonary or GI tract infections. Associated sinopulmonary infections are treated according to treatment protocols used for community-acquired respiratory tract infections in healthy persons, but prolonged treatment may be necessary.

Immunization with pneumococcal and other polysaccharide vaccines is important; however, not all patients are able to mount an immune response. Postvaccination IgG titers can be obtained to confirm the presence of an age-appropriate protective level of antipneumococcal IgG. Patients with common variable immunodeficiency (CVID) or more subtle specific antibody deficiencies may be unable to mount a response to polysaccharide antigens; therefore, pneumococcal vaccination in CVID patients is often ineffective.

Use of IGIV as replacement therapy is not indicated for selective IgAD per se. In selected circumstances in patients with concomitant SIgAD and selective IgG antibody deficiency who have recurrent or chronic high-grade sinopulmonary infections, a trial of IGIV may be given to see if a substantial clinical response occurs. Most patients with IgAD as part of CVID and/or with concomitant specific IgG antibody deficiency can safely receive intravenous (IV) or subcutaneous (SC) IgG replacement therapy.33,65

Patients with known or possible anti-IgA antibodies are still at increased risk of anaphylaxis or severe IgG-mediated reactions.

Precautions must be used in the administration of IV immunoglobulin and other blood products in patients with IgAD because IV immunoglobulin preparations and other blood products contain at least small amounts of IgA.

Vaccines, inactivated bacteria

Used to induce active immunity.


Pneumococcal vaccine 23-valent (PPV23; Pneumovax 23; Pnu-Imune 23)

Contains capsular polysaccharides of 23 pneumococcal types, which comprise 98% of pneumococcal disease isolates. For use in children >2 y and adults at increased risk of pneumococcal disease and its complications because of other underlying health conditions. Also benefits adults >65 y.

Adult

0.5 mL IM/SC
Following bone marrow transplant (use of PCV7 under study): One dose PPV23 at 12 mo and 24 mo following procedure

Pediatric

<2 years: Not recommended (see PCV7)
>2 years: Administer as in adults; PPV23 can be given to children >2 y and offers protection not covered with PCV7; can be given to children with newly recognized SIgAD
Serum can be obtained to determine if protective levels are achieved; if IgG pneumococcal antibody levels in the PCV7 remain low after children >2 y are given the PPV23, the authors give an additional PCV7; the authors see some children who have specific IgG pneumococcal antibody deficiency and lose immunologic memory (Sorensen, 1996)
Previously vaccinated with PCV7 vaccine, children >2 years, and adults with sickle cell disease, asplenia, immunocompromise, or HIV infection: 0.5 mL at age 2 y and then 2 mo after last dose of PCV7; revaccination with PPV23 administered 3-5 y after previous dose of PPV23 for children <10 y and, for children >10 y, every 3-5 y; revaccination should not be administered <3 y after previous PPV23 dose
Chronic illness: 0.5 mL in children >2 y and then 2 mo after last dose of PCV7; revaccination with PPV23

Effects decrease with immunosuppressive agents (eg, immunosuppressive doses of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents); globulin preparations may interfere with immune response to pneumococcal vaccine and reduce efficacy (do not administer within 3 mo of vaccine)

Documented hypersensitivity to vaccine or any component; active infection, Hodgkin disease, 10 d prior to or during treatment with immunosuppressive drugs or radiation; children <2 y (children <2 y do not respond satisfactorily to capsular types of 23 pneumococcal vaccine); pregnancy (safety of vaccine has not been evaluated; do not administer during pregnancy unless risk of infection is high)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Epinephrine injection (1:1000) must be immediately available in case of anaphylaxis; use caution in individuals who have had episodes of pneumococcal infection within preceding 3 y (preexisting pneumococcal antibodies may result in increased reaction to vaccine); may cause relapse in patients with stable idiopathic thrombocytopenia purpura


Pneumococcal 7-valent conjugate vaccine (PCV7; Prevnar)

Pneumococcal conjugate vaccine approved for infants and toddlers. Contains 7 purified capsular polysaccharides of S pneumoniae serotypes, accounting for 71% of infection among children <24 m, each coupled with a nontoxic variant of diphtheria toxin, CRM 197.
Licensed for use in infants and young children in Feb 2000. Recommended for children aged 2-23 mo and for children aged 24-59 mo who are at increased risk for pneumococcal disease (eg, with sickle cell disease, HIV infection, other immunocompromising or chronic medical conditions). Licensed for infants aged >6 wk.

Adult

Not recommended; see PPV23

Pediatric

0.5 mL IM at ages 2, 4, 6, and 12-15 mo

Effects may decrease with immunosuppressive agents (immunosuppressive doses of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents); pneumococcal 7-valent conjugate vaccine may increase effects of anticoagulant therapy; globulin preparations may interfere with immune response to pneumococcal vaccine and reduce efficacy (do not administer within 3 mo of vaccine)

Documented hypersensitivity to any component or diphtheria toxoid; severe or moderate febrile illness; infants or children with thrombocytopenia or coagulation disorder contraindicating IM injection (unless benefits outweigh risks)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Concurrent administration of PCV7 and PPV23 not recommended because safety and efficacy of concurrent vaccination have not been studied; epinephrine injection (1:1000) must be immediately available in the case of anaphylaxis; caution in individuals who have moderate or severe illness with or without fever, or delay vaccination until child has recovered

More on Immunoglobulin A Deficiency

Overview: Immunoglobulin A Deficiency
Differential Diagnoses & Workup: Immunoglobulin A Deficiency
Treatment & Medication: Immunoglobulin A Deficiency
Follow-up: Immunoglobulin A Deficiency
Multimedia: Immunoglobulin A Deficiency
References
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Further Reading

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Keywords

immunoglobulin A deficiency, IgA deficiency, immunodeficiency, selective IgA deficiency, SIgAD, selective immunoglobulin A deficiency, secretory IgA, primary antibody deficiencies, primary antibody deficiency, antibody deficiency, IgAD, IgG subclass deficiency, immunodeficiency disease, sinopulmonary infection, sinus infection, otitis media, stomach cancer, Giardia lamblia, G lamblia, Escherichia coli, E coli, Helicobacter pylori, H pylori, GI cancer, gastrointestinal disease, GI disease, Crohn disease, upper respiratory tract infection, lower respiratory tract infection, chronic diarrhea, transfusion complication, blood product reaction, adverse transfusion reaction, common variable immunodeficiency, CVID, TACI, TNF, tumor necrosis factor, forced vital capacity, FVC, bronchial hyperreactivity, BHR, autoimmune Addison disease, AAD, tTG, anti-tissue transglutaminase antibodies

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Contributor Information and Disclosures

Author

Rebecca Bascom, MD, MPH, Professor of Medicine, Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Pennsylvania State College of Medicine, Milton S Hershey Medical Center; Graduate Faculty Member, Pennsylvania State University College of Medicine and The Huck Institutes of the Life Sciences
Rebecca Bascom, MD, MPH is a member of the following medical societies: American College of Chest Physicians, American Public Health Association, American Thoracic Society, and Pennsylvania Thoracic Society
Disclosure: Nothing to disclose.

Coauthor(s)

Marina Y Dolina, MD, Consulting Physician, Lung, Sleep and Critical Care Consultants and Pulmonary Services, Wellspan Health System
Marina Y Dolina, MD is a member of the following medical societies: American College of Chest Physicians, American Medical Association, American Thoracic Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Melvin Berger, MD, PhD, Adjunct Professor of Pediatrics and Pathology, Case Western Reserve University; Senior Medical Director, Clinical Research and Development, CSL Behring, LLC
Melvin Berger, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Clinical Investigation, and Clinical Immunology Society
Disclosure: CSL Behring Salary Employment

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael R Simon, MD, MA, Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital
Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, and Society for Experimental Biology and Medicine
Disclosure: Secretory IgA, Inc. Ownership interest Board membership

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Michael A Kaliner, MD, Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy
Michael A Kaliner, MD 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 Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians
Disclosure: Abbott Consulting fee Consulting; Alcon Consulting fee Consulting; Glaxo Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering Consulting fee Consulting; Teva  Consulting; Meda Honoraria Speaking and teaching

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