An Update on Treatment Strategies for Common Variable Immunodeficiency

Drahomíra Detková, MD, PhD; Teresa Español, MD, PhD

Disclosures

Expert Rev Clin Immunol. 2009;5(4):381-390. 

In This Article

Abstract and Introduction

Abstract

Common variable immunodeficiency (CVID) is a primary immunological disease with variable severity, ranging from mild forms of infections to chronic progressive complications. The hallmark of this disorder is hypogammaglobulinemia due to an unknown molecular defect in immune regulation. The primary clinical manifestations are recurrent infections that may lead to structural damage of affected organs. Adequate intravenous immunoglobulin (Ig) therapy has dramatically changed the prognosis of this disorder, and the advent of home subcutaneous Ig therapy has further improved the quality of life of these patients. Aberrant T-cell functions predispose to autoimmune, inflammatory and lymphoproliferative complications, as well as malignancies in a variable percentage of CVID patients. Immunosuppressive anti-inflammatory therapies and chemotherapy are used, although always in conjunction with adequate replacement Ig therapy and adjunct antimicrobial prophylaxis. Any organ can be involved and therefore a multidisciplinary approach to the management of this disorder is essential.

Introduction

Common variable immunodeficiency (CVID) is a clinically and immunologically heterogeneous group of primary immunodeficiencies characterized by defective antibody production and susceptibility to recurrent infections.[1,2] CVID diagnostic criteria as defined by the Pan-American Group for Immunodeficiencies and the European Society for Immunodeficiencies include adults and children over the age of 2 years, serum IgG and IgA levels at least two standard deviations below the mean for the patient's age, failure to mount a significant specific antibody response to polysaccharide and protein antigens, and a ruling out of other known genetic or acquired causes of hypogammaglobulinemia.[1–4] In particular, normal or raised levels of serum IgM should alert to the possible existence of hyper-IgM syndromes instead of CVID. The exact prevalence of CVID is unknown and varies among different ethnicities. The estimated prevalence of CVID in the Caucasian population is 1 in 10–50,000. This disorder is the second most common primary immunodeficiency worldwide (after selective IgA deficiency) and the most common requiring immunoglobulin (Ig) replacement therapy.[5] Familial clustering of CVID, together with selective IgA deficiency, may be observed in up to 10% of patients.[2,6] Age at presentation and clinical manifestations are very variable.[7–9] Although CVID patients usually report onset of symptoms in the second or third decade of life, the disease may present at any age and, therefore, both pediatricians and adult healthcare practitioners should be aware of the existence of this disorder. Early diagnosis and timely treatment are the factors that most affect the prognosis of patients with this potentially life-threatening disorder.[7–11]

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