Immunoglobulin G Deficiency Follow-up

Updated: Mar 01, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Michael A Kaliner, MD  more...
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Follow-up

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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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. [36] 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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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. [5] 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

The patient and the patient's family should be taught how to recognize early signs and symptoms of infection so that the patient may obtain treatment as early as possible.

The patient should be advised to avoid crowded places and people who have active infections.

For patient education resources, see the Pneumonia Center, Lung and Airway Center, and Asthma Center, as well as Bacterial Pneumonia, Bronchitis, Asthma, Sinus Infection, and Myeloma.

A common data pool has been established in Europe as an Internet-based database for clinical and research data on patients with primary immunodeficiencies in order to facilitate epidemiological analyses, development of new diagnostic and therapeutic strategies, and the identification of novel disease-associated genes. [37]

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