eMedicine Specialties > Allergy and Immunology > Immunodeficiencies

Immunoglobulin G Deficiency: Follow-up

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
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
Contributor Information and Disclosures

Updated: Jul 9, 2009

Follow-up

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.

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.

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.

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.

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.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • A failure to use the correct method of testing or standardized age-related values could result in an incorrect diagnosis of IgG deficiency.
  • A failure to recognize the variations in normal and pathologic levels of total serum IgG and IgG subclass concentrations could result in complications and be a medicolegal pitfall.
  • Primary care physicians should be knowledgeable and trained well in order to provide an early diagnosis of IgG deficiency. The patient's major clinical problems should be addressed, and a thorough clinical workup should be completed.
  • In children, treatment must be started as soon as suggestive signs and symptoms are identified in order to prevent complications (eg, bronchiectasis). Adult patients with related symptoms should be seen frequently by their primary care physician until the diagnosis is either established or excluded.
  • Screening procedures are useful in the diagnosis of these diseases.
  • Patients with IgG deficiency (particularly those with low levels of IgG2) should be informed that pneumococcal or H influenzae type b vaccines that are prepared with isolated polysaccharide antigen might not provide sufficient immunity against these infections. Prospective studies confirmed a failure of response to soluble polysaccharide antigen in patients with high incidences of recurrent infections.
  • Rotation of the injection sites should be considered in patients who receive frequent injections.
  • Because transient IgG subclass deficiency is associated with a delayed development of antipolysaccharide antibody response, children with this deficiency should receive special care against possible infection, regardless of vaccination status.

Special Concerns

Additional education materials for patients, families, school personnel, and health professionals are available from the Immune Deficiency Foundation and the National Institute of Allergy and Infectious Disease.

 


More on Immunoglobulin G Deficiency

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

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Keywords

immune deficiency, immune globulin deficiency, IgG deficiency, IgG subclass deficiency, insufficient antibody production, gammaglobulin deficiency, hypogammaglobulinemia, immune deficiency, immunity, immunology, immune function, immunologic system interaction, autoimmune disorders, T-cell disease, B-cell dysfunction, complement deficiency, immunological disturbances, white blood cell diseases, WBC diseases, immunotherapy, intravenous immune globulin treatment, IVIG treatment, IV immunoglobulin treatment, common variable immunodeficiency, CVI, CVID, ataxia-telangiectasia, Sjogren syndrome, Sjogren's syndrome, X-linked agammaglobulinemia, X-LA, XLA, congenital agammaglobulinemia, transient hypogammaglobulinemia of infancy, Bruton’s, sinusitis, chronic sinusitis, recurrent sinusitis, tympanic membrane, effusion of middle ear, middle ear effusion

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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.

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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