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.
The cornerstone of therapy is immune globulin (IG) replacement, which 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 .
The treatment and health maintenance of patients with CVID will be discussed here, with an emphasis on adults. The clinical manifestations, diagnosis, and pathogenesis of this disorder, and issues particularly relevant to pediatric patients, are presented separately. (See "Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults" and "Common variable immunodeficiency in children" 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 some vaccines, IG therapy may be postponed, but these patients should be followed closely. (See '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]. The effectiveness of IG replacement in hypogammaglobulinemic patients was immediately apparent after this therapy became available, and so randomized controlled trials were never undertaken. 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 . In a large series of 2212 patients with CVID enrolled in a European primary immunodeficiency registry, patients receiving IVIG at doses yielding higher serum levels had fewer serious infections and days of hospitalization for primary immunodeficiency, compared with those with lower serum levels . However, treatment with IG does not entirely eliminate infections in most patients, and the sinopulmonary and gastrointestinal systems, in particular, remain susceptible. (See 'Sinusitis' below and 'Gastrointestinal infections' below.)