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Treatment and prognosis of common variable immunodeficiency

INTRODUCTION

Common variable immunodeficiency (CVID) is an immune disorder characterized by impaired B cell differentiation with hypogammaglobulinemia. The disorder is associated with a broad spectrum of clinical manifestations, including recurrent infections, chronic lung disease, gastrointestinal disease, and autoimmune disorders.

Immune globulin (IG) replacement therapy has dramatically altered the clinical course of CVID by reducing the burden of recurrent infections and subsequent complications. Management also involves vigilant monitoring for associated problems, such as pulmonary damage, gastrointestinal, autoimmune and granulomatous diseases, and malignancy [1].

The treatment and health maintenance of patients with CVID will be discussed here. The clinical manifestations, diagnosis, and pathogenesis of this disorder are presented separately. (See "Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults" and "Pathogenesis of common variable immunodeficiency".)

IMMUNOGLOBULIN REPLACEMENT THERAPY

The definition of common variable immunodeficiency (CVID) includes individuals with varying degrees of loss of antibody. For those with substantial impairments in antibody production and nonresponse to both protein and carbohydrate vaccines, immune globulin (IG) replacement is necessary. For subjects with higher levels of serum immunoglobulin G (IgG) and only minor impairments in response to carbohydrate vaccines, IG therapy may be postponed, but these patients should be followed closely. (See 'Utility in patients without infections or with isolated autoimmune disease' below.)

Impact of therapy — Ig replacement therapy reduces the number of infections and decreases antibiotic use and hospitalizations [2-4]. In one retrospective series of 50 patients treated with intravenous immune globulin (IVIG), the annual incidence of pneumonia decreased from 81 percent before treatment, to 35 percent on therapy, and the rate of hospitalization decreased from 89 to 46 percent [4]. The effectiveness of IG replacement in hypogammaglobulinemic patients was immediately apparent after this therapy became available, and so randomized controlled trials were never undertaken.

                                   

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Literature review current through: Jun 2014. | This topic last updated: May 19, 2014.
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