IgA and IgG Subclass Deficiencies Treatment & Management

Updated: Aug 14, 2014
  • Author: Terry W Chin, MD, PhD; Chief Editor: Harumi Jyonouchi, MD  more...
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Treatment

Medical Care

As noted in Medication, even if the patient cannot produce specific antibodies, the decision to treat with intravenous immunoglobulin (IVIg) is controversial. Aggressive use of antibiotics is required for recurrent respiratory tract infections, such as sinusitis, asthma, and bronchitis.

The use of prophylactic antibiotics (eg, during the winter months) has not been well studied but can be considered. Cohort studies indicate that this approach can be successful. [50] If conventional initial intervention with antibiotics is not successful, a trial of IVIg at 400-600 mg/kg for 6 months may be considered. For partial deficiencies (ie, specific antibody deficiency), IVIg can be stopped if no clinical response is observed.

In IgG subclass deficiency, most clinicians reserve IVIg for patients unable to make antibodies to both protein and polysaccharide antigens. Although reports mention a possible beneficial effect of decreased duration of bacterial infection in patients with IgG subclass deficiency, [51] an unpublished, blinded, and randomized trial of IVIg in IgG subclass deficiency showed that IVIg was not effective. However, cohort studies indicate a benefit, especially in those patients with fewer responses to pneumococcal polysaccharide challenge. [31]

Some advocate using IVIg if a patient aged 3 years or older does not respond to the unconjugated vaccine, especially if the titer or quantity to any 1 serotype (eg, type 3 polysaccharide, the most immunogenic) does not increase 2-fold.

Patients with a decreased ability to make antipolysaccharide antibodies should be immunized with polysaccharide-protein conjugate vaccines, such as H influenzae type b (HIB) with diphtheria-tetanus. The conjugated protein allows anti-HIB antibodies to develop, though 2 or 3 doses are usually required.

A conjugated pneumococcal vaccine is now licensed for use in the United States. Conjugated meningococcal vaccine (Menactra) is also now yet available in the United States. Sorensen et al (1998) showed that a significant percentage of children with specific antibody deficiency develop protective antibody levels to the conjugated pneumococcal vaccine (Prevnar) with a subsequently decreased rate of infections. [52] Patients with isolated IgG3 subclass deficiency have a similar dilemma as to whether IVIg is helpful. [53]

OM-85 BV has been used in Europe for over 20 years to stimulate mucosa-associated lymphoid tissue and up-regulate the TH-1 response. One study did not find any increased induction of autoantibodies in IgA-deficient patients. [54] The authors did not comment on any beneficial effect in these patients. More specific therapy may become available if clinical studies on interleukin 21 confirm potential therapy in patients with common variable and selective IgA deficiencies. [55]

Aggressive treatment of underlying allergies and/or asthma may help reduce the frequency and/or severity of recurrent respiratory tract infections, such as sinusitis and bronchitis.

Conventionally treat associated autoimmune diseases. Nothing indicates that patients with a concomitant specific IgA deficiency do worse than those without any immunodeficiency.

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Surgical Care

A few patients with chronic upper or lower respiratory infections and subsequent structural changes may need strategic, long-term, broad-spectrum antibiotics, in addition to chest physiotherapy and sinus surgery.

Although many patients benefit from the placement of tympanostomy tubes to manage recurrent otitis media and/or they might undergo endoscopic sinus surgery for chronic sinusitis, the importance of aggressive medical therapy for the underlying immunodeficiency and its accompanied allergic condition cannot be overemphasized.

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Consultations

Consultation with a surgeon may be needed for patients with chronic infections of the upper or lower respiratory tracts. Chronic sinusitis may require various ear, nose, and throat (ENT) procedures to promote drainage.

A rheumatologist, allergist/immunologist, or both may be required because of the various autoimmune and allergic diseases present with increased frequency in B-cell disorders.

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Diet

Gluten-free and other restricted diets have been tried but are ineffective in these disorders when chronic diarrhea is present.

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Activity

Encourage normal activity.

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