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Treatment and prognosis of common variable immunodeficiency

Sam Ahn, MD
Charlotte Cunningham-Rundles, MD, PhD
Section Editor
E Richard Stiehm, MD
Deputy Editor
Anna M Feldweg, MD


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 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 [1].

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".)


The definition of CVID includes individuals with varying degrees of loss of antibody. For those with substantial impairments in immune globulin production (eg, generally two standard deviations below the normal range for immunoglobulin G [IgG]) and nonresponse to both protein and polysaccharide vaccines, immune globulin replacement is necessary. For subjects with higher levels of serum IgG and only minor impairments in response to some vaccines, immune globulin replacement therapy may be postponed, but these patients should be followed closely. (See 'Patients without infections or with isolated autoimmune disease' below.)

Overview of administration — Immune globulin replacement therapy may be administered either intravenously or subcutaneously. A typical approach is to begin therapy with intravenous immune globulin (IVIG), although one can also start with subcutaneous immune globulin (SCIG), with an initial loading regimen. If the intravenous route is used to initiate therapy, the subcutaneous route may be substituted after two or more months on IVIG, if this is preferred. We occasionally start patients with very poor venous access on SCIG from the outset. A more detailed discussion of the characteristics and safety of various preparations of IVIG, how to initiate therapy with IVIG or SCIG, and how to convert from IVIG to SCIG, is found separately. (See "Immune globulin therapy in primary immunodeficiency" and "Subcutaneous and intramuscular immune globulin therapy".)

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Literature review current through: Nov 2017. | This topic last updated: Mar 15, 2017.
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