Immunoglobulin G Deficiency Follow-up

  • Author: Robert Y Lin, MD; Chief Editor: Michael A Kaliner, MD   more...
 
Updated: Sep 28, 2010
 

Further Inpatient Care

  • Patients should be monitored at regular intervals to be sure potential complications, including acquisition of potential bloodborne infections, have not occurred. In addition, immunologic and pulmonary status of patients with primary immune deficiency diseases should be monitored by the physician. In most cases, doses of IgG are individualized to achieve optimal freedom from acute infection.
  • Low IgG levels in patients with IgG deficiency are not necessarily associated with deficiencies of antibody responses to immunization with vaccines (eg, tetanus toxoid). Only a small percentage of patients have impaired responses to certain vaccinations. Therefore, measuring antibody levels after vaccination, particularly pneumococcal polysaccharide vaccine (Pneumovax) and Haemophilus vaccine, is crucial before initiating IgG replacement therapy. If the response to vaccination with the above polysaccharide vaccines is inadequate, revaccination with protein-conjugated polysaccharide vaccines, heptavalent pneumococcal conjugate vaccine (Prevnar) and Haemophilus vaccine, is indicated.
  • Serological tests for specific infections may be unreliable in patients with primary immune deficiency diseases, and false-positive results (ie, positive IgG titers to EBV, hepatitis A virus, and hepatitis B surface antigen) may result from antibodies contained in IgG preparations. Therefore, physicians may want to use nucleic acid amplification tests (eg, polymerase chain reaction [PCR]) to document the presence or absence of such infections before beginning therapy.
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Further Outpatient Care

  • Careful health care may reduce the number of infections, which are common problems. Chest physiotherapy may be needed to treat such infections.
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Inpatient & Outpatient Medications

  • Physicians should stress the importance of good hygiene, good nutrition, and adherence to recommendations for therapy and prevention of infections. The long half-life of IVIG is advantageous, and this type of therapy provides several weeks of protection against related microorganisms. These results have also been observed in patients with IgG subclass deficiency, X-linked agammaglobulinemia, and common variable immunodeficiency (CVID).
  • Many patients also benefit from the use of prophylactic or chronic treatment regimens of oral antibiotics. Patients with chronic bronchitis or obstructive pulmonary disease may benefit from bronchodilators, topical corticosteroids, or both. Patients with chronic sinus disease may benefit from expert evaluation and management by ENT specialists; this may include surgery.
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Complications

  • Intravenous immunoglobulin (IVIG), whole blood transfusions, and packed red blood cell transfusions are contraindicated in patients who have IgA deficiency. In these patients, the development of anti-IgA antibodies has been associated with both nonhemolytic transfusion reactions and anaphylaxis. If blood transfusions are required in a patient with IgA deficiency, washed red blood cell transfusions should be given.
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Prognosis

Patients on appropriate IgG replacement regimens can expect freedom from serious acute bacterial infections. However, patients with CVID are at increased risk of autoimmune disease and malignancy, particularly mucosa-associated lymphoid tissue (MALT) lymphomas.[3] In addition, freedom from acute infections or exacerbations does not necessarily imply a lack of progression of bronchiectasis or other chronic conditions.

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Patient Education

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Contributor Information and Disclosures
Author

Robert Y Lin, MD  Professor, Department of Medicine, Medical Advisor, Department of Case Management/Utilization Review, New York Medical College; Chief, Allergy and Immunology Section, St Vincent's Catholic Medical Centers, St Vincent's of Manhattan

Robert Y Lin, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology and American Federation for Medical Research

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Janet Vafaie, MD  Private Practice

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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 Management position

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St 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: Alcon Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering/Merck Consulting fee Consulting; Teva Consulting fee Consulting; Meda Honoraria Speaking and teaching; Ista Consulting

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Immunoglobulin G deficiency. Schematic representation of an immunoglobulin G molecule. CH indicates constant region of heavy chain; CL, constant region of light chain; VH, variable region of heavy chain; and VL, variable region of light chain.
Immunoglobulin G deficiency. Human immunoglobulin G subclasses.
Immunoglobulin G deficiency. Changes in serum immunoglobulin G concentrations during infancy and childhood.
Immunoglobulin G deficiency. Schematic representation of the molecular interaction of CD40 on B cells, with CD40L on T cells involved in the switch from immunoglobulin M to immunoglobulin G.
 
 
 
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