What is Common Variable Immunodeficiency (CVID)?

From the Guidelines

Common Variable Immunodeficiency (CVID) is a disorder characterized by impaired immune function, recurrent infections, and increased risk of autoimmune disorders and certain cancers, requiring aggressive management with antimicrobials, IgG replacement, and careful attention to pulmonary status. This condition is marked by low levels of immunoglobulins (antibodies) in the blood, particularly IgG, IgA, and sometimes IgM, despite having normal B cell counts 1. Patients with CVID typically experience recurrent bacterial infections, especially in the respiratory and gastrointestinal tracts, along with an increased risk of autoimmune disorders and certain cancers.

Key Characteristics of CVID

• Low levels of immunoglobulins (antibodies) in the blood
• Recurrent bacterial infections, especially in the respiratory and gastrointestinal tracts
• Increased risk of autoimmune disorders and certain cancers
• Normal B cell counts

Management of CVID

• Aggressive management with antimicrobials, IgG replacement, and careful attention to pulmonary status is essential 1
• IgG replacement therapy, typically administered intravenously (IVIG) or subcutaneously (SCIG), to provide the antibodies patients cannot produce themselves
• Prophylactic antibiotics may be prescribed for patients with recurrent infections
• Prompt treatment of infections with appropriate antibiotics is essential

Complications of CVID

• Infectious lung disease occurs in the majority of patients with a clinical presentation very similar to that of allergic asthma
• Noninfectious chronic pulmonary disease occurs in nearly 30% of patients and is associated with reduced survival
• Bronchiectasis is the most common pulmonary complication of CVID, occurring in 10% to 20% of patients
• Gastrointestinal complications, such as chronic gastritis, lymphoid nodular hyperplasia, and inflammatory bowel disease, occur in approximately 20% to 25% of patients
• Autoimmune diseases, such as autoimmune cytopenias, occur in approximately 20% of patients 1

From the Research

Definition and Characteristics of Common Variable Immunodeficiency (CVID)

• CVID is a primary immunological disease with variable severity, characterized by hypogammaglobulinemia due to an unknown molecular defect in immune regulation 2.
• It is a heterogeneous group of primary antibody deficiency disorders, characterized by recurrent infection and inflammatory, granulomatous, and autoimmune complications 3.
• CVID can develop at any age without gender predominance, and its prevalence varies widely worldwide 4.

Clinical Manifestations of CVID

• The primary clinical manifestations are recurrent infections that may lead to structural damage of affected organs 2.
• Patients with CVID can also develop autoimmune diseases, lymphoproliferation, malignancies, and granulomatous lesions 4.
• Autoimmunity can be the only clinical manifestation of CVID at the time of diagnosis and may even develop prior to hypogammaglobulinemia 4.
• Clinical features of CVID patients can include abnormal liver biochemistries, nodular regenerative hyperplasia (NRH), or liver cirrhosis and its complications 4.

Diagnosis and Treatment of CVID

• The diagnosis of CVID is largely based on the criteria established by European Society for Immunodeficiencies and Pan-American Group for Immunodeficiency (ESID/PAGID) 4.
• Replacement therapy with immunoglobulin (Ig) and anti-infection therapy are the primary treatment regimen for CVID patients 4.
• Early diagnosis and appropriate treatment with intravenous and subcutaneous immunoglobulins can reduce the frequency of infections and their potential complications 5.
• A multidisciplinary approach to the management of CVID is essential, as any organ can be involved 2.

Prognosis and Complications of CVID

• The prognosis of CVID varies widely, and delays in diagnosis can lead to serious and irreversible complications 5.
• CVID patients are at an increased risk of developing cancer and lymphoma 6.
• Inflammatory and autoimmune diseases, such as lymphadenopathy, splenomegaly, autoimmune cytopenias, enteropathy, and granulomatous disease, can also occur in CVID patients 6.