Immunoglobulin A Deficiency Follow-up: Deterrence/Prevention, Complications, Prognosis

Sections

Immunoglobulin A Deficiency

Sections Immunoglobulin A Deficiency
Overview
Presentation
- History
- Physical
- Causes
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DDx
Workup
Treatment
Medication
Follow-up
Questions & Answers
Media Gallery
References

Follow-up

Deterrence/Prevention

See the list below:

Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued.
Secondary prevention relies primarily on vaccination to increase specific IgG and IgM (see Medication). In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi.
Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed).

Complications

See the list below:

Severe anaphylactic reactions to blood products
Bronchiectasis
Recurrent sinopulmonary infections
Chronic diarrhea
Severe otitis media resulting in hearing loss; case reports of deaths
Malabsorption syndrome
Growth retardation secondary to malabsorption and chronic infection

Prognosis

See the list below:

In children aged 6 months to 4 years, IgAD may be transient and resolve permanently by age 5 years; in others, the syndrome may progresses to common variable immunodeficiency (CVID).
Adults with selective immunoglobulin A deficiency (SIgAD) are often asymptomatic; however, up to 90% have frequent bacterial respiratory tract infections.

Patient Education

See the list below:

Educate patients to recognize early signs of respiratory tract infections, such as increased phlegm, discolored phlegm, cough, or dyspnea.

Questions

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Media Gallery

Chest radiograph of a 50-year-old man with immunoglobulin A deficiency and severe bilateral pneumonia. He also had congenital heart disease. Serum immunoglobulin G and immunoglobulin M levels were normal.
Lateral chest radiograph of a 50-year-old man with immunoglobulin A deficiency and severe bilateral pneumonia.
Portable chest radiograph of a 50-year-old man with acute respiratory distress syndrome as a complication of severe bilateral pneumonia. The patient died from respiratory failure 2 days after this x-ray film was taken.

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Author

Marina Y Dolina, MD Consulting Physician, Wellspan Lung, Sleep and Critical Care Consultants and Pulmonary Services, York Hospital, 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, Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

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 Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Michael R Simon, MD, MA Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Professor, Department of Internal Medicine, Oakland University William Beaumont 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, Society for Experimental Biology and Medicine

Disclosure: Have a 5% or greater equity interest in: Secretory IgA, Inc. ; siRNAx, Inc.<br/>Received income in an amount equal to or greater than $250 from: siRNAx, Inc.

Chief Editor

Michael A Kaliner, MD Clinical Professor of Medicine, George Washington University School of Medicine; 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, Association of American Physicians

Disclosure: Nothing to disclose.

Additional Contributors

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, Clinical Immunology Society

Disclosure: Received salary from CSL Behring for employment; Received ownership interest from CSL Behring for employment; Received consulting fee from America''s Health insurance plans for subject matter expert for clinical immunization safety assessment network acvtivity of cdc.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Bettina C Hilman, MD, to the development and writing of this article.

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