Conditions / Common Variable Immune Deficiency
Common Variable Immune Deficiency
Overview
Common Variable Immune Deficiency (CVID) is a primary immunodeficiency disorder characterized by low levels of antibodies (immunoglobulins), which makes it harder for the immune system to fight infections. Unlike other immune deficiencies that present early in life, CVID can appear at any age—most commonly in late childhood or early adulthood. People with CVID experience frequent infections, especially in the respiratory and gastrointestinal tracts, and may also develop autoimmune conditions or gastrointestinal disorders. Though it is a lifelong condition, early diagnosis and treatment with immunoglobulin replacement therapy can greatly improve health and quality of life.
Symptoms
Symptoms of CVID vary widely from person to person and may range from mild to severe. Common signs include:
• Frequent infections: Such as sinusitis, bronchitis, pneumonia, and ear infections.
• Chronic cough or lung damage: From repeated respiratory infections (can lead to bronchiectasis).
• Digestive issues: Including chronic diarrhea, abdominal pain, or weight loss.
• Fatigue: Often due to chronic inflammation or recurrent illness.
• Swollen lymph nodes or enlarged spleen.
• Autoimmune problems: Such as immune thrombocytopenia (low platelets), hemolytic anemia, or rheumatoid-like symptoms.
When to see a doctor
Consult a healthcare provider if you or a loved one experience:
• Repeated infections, especially of the lungs, sinuses, or ears.
• Illnesses that are unusually severe or slow to resolve.
• Chronic diarrhea or unexplained weight loss.
• Fatigue that doesn’t improve with rest.
• A personal or family history of immune disorders or autoimmune diseases.
Seek immediate medical attention for:
• High fever with no clear source.
• Severe shortness of breath or chest pain.
• Signs of systemic infection (e.g., confusion, rapid heart rate, or low blood pressure).
Causes
The exact cause of CVID is unknown in most cases, but it is believed to involve both genetic and immune system abnormalities:
• Impaired B-cell function: B cells are present but fail to mature properly and produce antibodies.
• Genetic mutations: Some cases are linked to inherited mutations, though not all patients have a known genetic cause.
• Dysregulated immune response: May involve other immune cells, contributing to autoimmune symptoms.
Risk Factors
You may be more likely to develop CVID if:
• There is a family history of primary immunodeficiency disorders.
• You are in late childhood or early adulthood: The most common age range for diagnosis.
• You have unexplained, recurring infections that don’t respond well to standard treatments.
Complications
If not properly managed, CVID can lead to a range of complications, including:
• Permanent lung damage: Such as bronchiectasis from repeated infections.
• Autoimmune disorders: Where the immune system mistakenly attacks the body.
• Gastrointestinal problems: Including inflammatory bowel disease or malabsorption.
• Increased risk of certain cancers: Especially lymphoma and gastrointestinal cancers.
• Chronic fatigue and decreased quality of life.
Medical Risk
Though there is no cure for CVID, treatment can be highly effective. The main goals are to prevent infections, manage symptoms, and monitor for complications. Standard treatment includes:
• Immunoglobulin (IgG) replacement therapy: Administered through IV or subcutaneous injections on a regular schedule.
• Antibiotics: To treat and sometimes prevent infections.
• Management of autoimmune conditions: With medications such as corticosteroids or immunosuppressants.
• Regular monitoring: Including blood work, imaging, and lung function tests.
Potential risks include:
• Side effects from Ig therapy: Such as headaches, flu-like symptoms, or injection-site reactions.
• Incomplete response to treatment: Some patients continue to have infections or autoimmune issues despite therapy.
• Delayed diagnosis: Which may result in irreversible damage, especially to the lungs or gut.
With early diagnosis, regular treatment, and careful monitoring, most people with CVID can lead active, fulfilling lives.
Treatments