How is common variable immunodeficiency (CVID) treated?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

A major component of medical care is anti-infective and prevention of further infectious episodes by regular infusion of human immunoglobulin and antimicrobial therapy. Patients with autoimmune manifestations may require immunosuppressive therapy.

Immunoglobulin replacement is intravenously administered on a regular basis, as follows:

  • Tailor dose and frequency to the Ig trough levels and to clinical symptoms

  • Measure serum IgG level before each infusion, and accordingly adjust the dose of IVIG

  • Maintain trough serum IgG concentrations at 400–500 mg/dL in adults

  • For most patients, a dose of 400-600 mg/kg every 3–4 weeks suffices to reduce the frequency of infection

  • Some patients with chronic lung disease require up to 600–800 mg/kg per month

  • Once established on a regular regimen, IVIG can be administered at home

Subcutaneous infusion of Ig (SCIG) is an alternative method for patients with difficult venous access or for those who experience serious side effects from IVIG.

Anti-infective treatment is as follows:

  • Infections should be treated early with full doses of antimicrobial agents

  • Whenever possible, narrow-spectrum drugs should be used on the basis of microbial sensitivity testing

  • Prophylactic antibiotics should be avoided

  • Antiviral agents may be useful in some patients with persistent or severe viral infections

Some patients with a severe autoimmune process may require immunosuppressive therapy with one of the following:

  • Systemic corticosteroids

  • Cyclosporin A

  • Anti-CD20 monoclonal antibody (rituximab)

  • Anti-TNF monoclonal antibody (infliximab)

See Treatment and Medication for more detail.


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