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Common variable immune deficiency
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Common variable immune deficiency

Common variable immune deficiency (CVID) is an immune system disorder that leaves individuals highly susceptible to recurrent infections due to an inability to produce sufficient antibodies.

Overview

CVID impairs the immune system, making people highly susceptible to bacteria and, more rarely, viruses. Recurrent infections, particularly in the ears, sinuses, and lungs, are common. Chronic lung disease can develop over time. Affected individuals may also experience gastrointestinal inflammation, leading to diarrhea and weight loss, as well as enlarged lymph nodes (lymphadenopathy) or an enlarged spleen (splenomegaly).

Autoimmune and Cancer Risks

Approximately 25 percent of people with CVID develop an autoimmune disorder, where the immune system attacks the body's own tissues. Common examples include:

  • Immune thrombocytopenia: A bleeding disorder caused by a decrease in platelets.
  • Autoimmune hemolytic anemia: Premature destruction of red blood cells.
  • Rheumatoid arthritis.

Individuals with CVID also have an increased risk of developing certain cancers, including lymphoma and, less frequently, stomach (gastric) cancer.

Causes

In about 90 percent of cases, the cause is unknown, though it is likely a combination of environmental and genetic factors. In the remaining 10 percent, mutations in genes such as TNFRSF13B are involved. These genes are essential for the development and function of B cells—specialized white blood cells that produce antibodies (immunoglobulins). Dysfunctional B cells cannot produce sufficient antibodies, making it difficult to fight infections or respond to vaccines.

Inheritance

Most cases are sporadic. In rare instances, CVID can be inherited in an autosomal recessive or autosomal dominant pattern. Not everyone who inherits a mutation associated with CVID will develop the disease, suggesting that additional genetic or environmental factors are required.

Frequently Asked Questions

Patients are most vulnerable to recurrent ear, sinus, and lung infections, as well as gastrointestinal infections that cause diarrhea. Practical measures include staying up to date on vaccinations (influenza, pneumococcal, COVID‑19), using prophylactic antibiotics when prescribed, practicing rigorous hand hygiene, avoiding crowded indoor settings during peak infection seasons, and seeking prompt medical care for any new respiratory or gastrointestinal symptoms. How is CVID distinguished from other primary immunodeficiencies during the diagnostic work‑up?::The hallmark is markedly low IgG, IgA, and often IgM levels with poor vaccine responses. A full immunoglobulin panel, flow cytometry to assess B‑cell subsets, and a detailed infection history are performed. Genetic testing for known CVID‑associated genes (e.g., TNFRSF13B) is done when the clinical picture is atypical or when family history suggests a hereditary pattern. What therapies are available to raise antibody levels in CVID patients, and how effective are they in preventing infections?::Intravenous or subcutaneous immunoglobulin replacement (IVIG/SCIG) is the cornerstone of treatment, providing the missing antibodies and reducing infection frequency by 50–80 %. Doses are tailored to maintain trough IgG levels above 6 g/L. In patients with severe autoimmune manifestations, additional immunosuppressive agents (e.g., rituximab) may be added, but the primary goal remains adequate immunoglobulin replacement. What warning signs indicate that a CVID patient might be developing an autoimmune complication or lymphoma?::New or worsening lymphadenopathy, unexplained weight loss, night sweats, persistent fevers, or a sudden rise in inflammatory markers (e.g., ESR, CRP) should prompt evaluation. A complete blood count with differential, imaging studies (CT or PET‑CT), and, if indicated, a lymph node biopsy are used to assess for lymphoma. Early detection of autoimmune cytopenias (e.g., thrombocytopenia, anemia) also requires prompt hematology referral.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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