Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (usually called CVID) is a long-term problem of the immune system. In CVID, the body does not make enough protective proteins called antibodies (also named immunoglobulins). Because of this, the body cannot fight germs well, and the person gets many infections again and again.[1] In most people with CVID, the blood level of IgG is low, and IgA and/or IgM are also low. These are three main kinds of antibodies. When they are low, vaccines do not work properly, and infections are harder to clear. Doctors must also rule out other reasons for low antibodies before they say it is CVID.[2]

Common variable immunodeficiency is a long-term disease where the body cannot make enough healthy antibodies (immunoglobulins) to fight germs. People with CVID get infections again and again, especially in the ears, sinuses, chest, and gut. They may also have problems like diarrhea, weight loss, swollen glands, liver disease, or autoimmune problems such as low blood counts. CVID is usually lifelong, but with good treatment many people can live active lives. The main goal of care is to replace missing antibodies, prevent infections, and treat complications early.

CVID can start in childhood, teenage years, or in adults. Many people are not diagnosed until their 20s or 30s, even if they had infections earlier. It is a lifelong condition, but with proper care many people can live active lives.[3]

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Other names for common variable immunodeficiency

CVID has a few other names or terms that doctors may use:

• Common variable immune deficiency – same meaning, just slightly different wording.[4]

• Common variable immunodeficiency disorders (CVID disorders) – used when doctors talk about the whole group of slightly different but related problems.[5]

• Primary antibody deficiency with hypogammaglobulinemia – this means a main (primary) immune problem where antibodies are low.[6]

All of these names point to the same basic idea: a common, mixed-type problem where antibody levels are low and infections happen often.[7]

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Types of common variable immunodeficiency

Doctors think CVID is not one single disease but a group of related conditions. There are different ways to talk about “types.”

1. Types based on gene changes (genetic types)

Researchers have found several gene changes linked to CVID. These are sometimes called CVID1, CVID2, and so on. Each type is tied to a different gene that affects B-cell function or antibody production.[8]

• CVID1 (ICOS-related) – a change in the ICOS gene that affects how immune cells talk to each other.

• CVID2 (TACI-related) – a change in the TACI gene, which helps B cells survive and switch antibody types.

• CVID3 (CD19-related) – a change in the CD19 gene, important for how B cells respond to signals.

• CVID4 (BAFF-receptor–related) – a change in the BAFF-receptor gene, which helps B cells stay alive.

• CVID5 (CD20-related) – a change in CD20, a protein on B cells.

• CVID6 (CD81-related) – a change in CD81, another helper protein on B cells.

These genetic types are found only in a minority of people with CVID. In many patients, no single clear gene change is found.[9]

2. Types based on main health problems (clinical patterns)

Doctors also divide CVID into groups based on what health problems are most important for that person:

• “Infection-only” CVID – people mainly have repeated sinus, ear, chest, or gut infections.

• CVID with autoimmunity – people have immune attacks on their own body, such as low platelets, low red cells, thyroid disease, or joint problems.[10]

• CVID with lymphoproliferation or granulomas – people have big lymph nodes, large spleen, or lumps of immune cells in organs.

• CVID with mainly gut or liver disease – people have long-term diarrhea, weight loss, or chronic liver problems.

These patterns help doctors guess which organs need the closest follow-up.

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Causes of common variable immunodeficiency

For most people with CVID, there is no single clear cause. It seems to come from a mix of genes and environment. Below are 20 known or suspected causes and contributing factors.

1. Inherited changes in immune genes – Some people inherit changes in genes that control B-cell growth and antibody production. These changes can be passed down in families and raise the risk of CVID.[11] [1]

2. New (spontaneous) gene mutations – In some people there is no family history, but a new change in an immune gene appears in that person and may lead to CVID-like problems.[12] [2]

3. Problems with B-cell development – B cells may not mature from early cells into fully working antibody-making cells. This failure reduces all antibody types.[13] [3]

4. Defects in class-switch recombination – B cells should “switch” from making IgM to IgG, IgA, or IgE. Faults in this switching step can lead to low IgG and IgA, as seen in many CVID patients.[14] [4]

5. Reduced memory B cells – Many people with CVID have low numbers of memory B cells, which normally remember past infections and vaccines. This makes vaccine responses weak or absent.[15] [5]

6. Abnormal T-cell help – T helper cells support B cells. In CVID, T cells can be low in number or function poorly, so B cells do not get the right help to make antibodies.[16] [6]

7. Disordered immune signaling molecules – Signals like BAFF and APRIL help B cells live and mature. Problems in these signal paths can disturb B-cell survival and antibody production.[17] [7]

8. Family history of primary immunodeficiency – Having close relatives with CVID or other antibody problems raises the chance of similar immune defects, suggesting shared genes.[18] [8]

9. Certain HLA (tissue type) patterns – Some tissue-type patterns in the major histocompatibility complex (MHC) seem more frequent in CVID, which may change how the immune system responds.[19] [9]

10. Unknown environmental triggers – In many people the exact “trigger” is not known. Infections or other exposures may act together with genes to push the immune system toward failure.[20] [10]

11. Previous serious infections – Some people are first noticed to have CVID after difficult infections. It is not clear if infections cause CVID or just reveal an immune weakness that was already there.[21] [11]

12. Abnormal gut immune responses – The gut immune system may be over-active in some ways and weak in others. This can lead to chronic gut inflammation and poor antibody responses.[22] [12]

13. Autoimmune processes – In some people, the immune system not only fails to protect but also attacks its own cells. This autoimmunity may share roots with the antibody defect.[23] [13]

14. Lymphoid tissue abnormalities – The organs that make and store immune cells (lymph nodes, spleen, gut lymph tissue) may grow in a strange way and may not support normal B-cell function.[24] [14]

15. Gene changes affecting cell death (apoptosis) – Some gene problems can make B cells die too early or survive in a damaged state, instead of maturing in a healthy way.[25] [15]

16. Defects in NF-κB signaling – NF-κB is a key pathway inside immune cells. Faults in this pathway have been linked to CVID-like conditions in some families.[26] [16]

17. Interplay with other immune defects – Some people have mixed patterns, such as low antibodies plus low T-cell numbers (late-onset combined immunodeficiency). This overlap can share similar genetic bases.[27] [17]

18. Possible epigenetic changes – Changes in how genes are switched on and off (without changing the DNA code) may also affect antibody production, although this is still being studied.[28] [18]

19. Age-related immune changes – In adults, natural changes in the immune system with age may interact with genetic risk to reveal CVID in later life.[29] [19]

20. Unknown or multifactorial causes – For many patients, doctors simply cannot find the exact cause. Most experts think several small gene and environment factors act together to produce CVID.[30] [20]

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Symptoms and signs of common variable immunodeficiency

Symptoms can be very different from one person to another. Here are 15 common ones.

1. Frequent sinus infections – Many people have repeated sinusitis with blocked nose, pain in the face, and yellow or green mucus because the body cannot clear bacteria well.[31] [1]

2. Ear infections (otitis media) – Repeated middle-ear infections cause ear pain, fever, and sometimes hearing problems, especially in children and teens.[32] [2]

3. Chest infections and pneumonia – People with CVID often get bronchitis and pneumonia, with cough, fever, chest pain, and shortness of breath.[33] [3]

4. Bronchiectasis (permanent airway damage) – Repeated lung infections can damage airway walls, leading to widened, scarred tubes, chronic cough, and thick sputum.[34] [4]

5. Gut infections and diarrhea – Many people have long-lasting or repeated diarrhea, belly pain, and weight loss, often from infections or immune-related gut inflammation.[35] [5]

6. Unexplained weight loss – Ongoing infections and poor nutrient absorption from the gut can cause weight loss and poor growth in children.[36] [6]

7. Swollen lymph nodes – Lymph nodes in the neck, armpits, or groin can become enlarged because the immune system is constantly activated or grows in an abnormal way.[37] [7]

8. Enlarged spleen (splenomegaly) – The spleen may become big because it is overworking or because immune cells are clustering there.[38] [8]

9. Autoimmune low platelets (ITP) – The immune system may attack platelets, causing easy bruising, nosebleeds, or bleeding gums.[39] [9]

10. Autoimmune hemolytic anemia – The immune system may break down red blood cells, causing pale skin, tiredness, dark urine, and sometimes yellow eyes.[40] [10]

11. Joint pain and arthritis-like symptoms – Some people get aching or swollen joints due to autoimmune inflammation.[41] [11]

12. Skin problems – Rashes, dry skin, or small red spots can happen due to infections, autoimmunity, or inflammation in blood vessels.[42] [12]

13. Chronic fatigue – Many people feel very tired even between infections because the body is always trying to fight germs and inflammation.[43] [13]

14. Liver problems – Some patients develop liver disease, such as nodular regenerative hyperplasia or portal hypertension, which can cause fluid build-up in the belly or abnormal blood tests.[44] [14]

15. Higher risk of some cancers – Over time, there is a higher risk of certain cancers, especially lymphoma and stomach cancer, likely because of long-term immune activation and chronic infection.[45] [15]

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Diagnostic tests and examinations for common variable immunodeficiency

To diagnose CVID, doctors combine the story, physical exam, and several tests. They must show low antibodies, poor vaccine response, and exclude other causes.[46]

Physical exam tests

1. General physical examination – The doctor looks at the whole body: weight, height, any signs of poor growth, pallor (pale skin), rashes, or bruises. This helps show if infections or autoimmune problems have been going on for a long time.[47] [1]

2. Vital signs check – Temperature, heart rate, breathing rate, and blood pressure are measured. Fever or fast breathing can show active infection, while low blood pressure may suggest a serious infection.[48] [2]

3. Respiratory system examination – The doctor listens to the lungs with a stethoscope, looking for crackles or wheezes that may suggest pneumonia or bronchiectasis in people with repeated chest infections.[49] [3]

4. Abdominal and spleen examination – By gently pressing on the belly, the doctor can feel if the liver or spleen is enlarged, which is common in some CVID patients.[50] [4]

Manual tests (bedside hands-on checks)

5. Lymph node palpation – The doctor uses their hands to feel lymph nodes in the neck, armpits, and groin. Enlarged, firm nodes can signal chronic infection, immune activation, or risk of lymphoma.[51] [5]

6. Sinus tenderness test – Gentle pressure over the forehead and cheekbones can show tenderness that suggests sinus infection, which is common in CVID.[52] [6]

7. Joint examination – The doctor moves and feels the joints, looking for swelling, warmth, or pain, which may suggest autoimmune arthritis related to CVID.[53] [7]

Lab and pathological tests

8. Serum immunoglobulin levels (IgG, IgA, IgM) – This is the key blood test. IgG is clearly low, and IgA and/or IgM are also low, usually at least two standard deviations below the normal level for age.[54] [8]

9. Specific antibody response to vaccines – Doctors measure antibody levels after vaccines (such as tetanus or pneumococcal vaccines). In CVID, these responses are weak or absent, even though the person was fully vaccinated.[55] [9]

10. Complete blood count (CBC) – This blood test looks at red cells, white cells, and platelets. It can show anemia, low platelets from autoimmunity, or abnormal white cell counts.[56] [10]

11. Lymphocyte subset analysis (flow cytometry) – This test counts types of lymphocytes (B cells, T cells, NK cells) and may show low memory B cells or low CD4 T cells, patterns that support CVID.[57] [11]

12. Inflammatory markers (CRP, ESR) – These blood tests help detect active infection or ongoing inflammation in the body.[58] [12]

13. Liver function tests – Blood tests of liver enzymes and proteins can show liver injury or disease, which can occur in some CVID patients.[59] [13]

14. Stool tests for infection and inflammation – In people with chronic diarrhea, stool tests look for germs and markers of inflammation to understand gut involvement.[60] [14]

15. Genetic testing panels for primary immunodeficiency – A blood sample is checked for known gene changes linked to CVID and related immune problems. A positive result can confirm the underlying cause in some patients, but a normal result does not rule out CVID.[61] [15]

Electrodiagnostic tests

16. Electrocardiogram (ECG) – An ECG checks heart rhythm and strain. In CVID, it is not a main diagnostic test, but it may be used in people with severe lung disease, chest infections, or drug side effects to make sure the heart is safe.[62] [16]

17. Nerve conduction studies (if needed) – Rarely, when there are signs of nerve damage (for example from autoimmune disease or vitamin lack), nerve tests are done to see how fast signals move along the nerves.[63] [17]

Imaging tests

18. Chest X-ray – A simple X-ray of the chest can show pneumonia, long-standing lung damage, or enlarged heart or vessels. It is often the first imaging test in someone with repeated chest infections.[64] [18]

19. High-resolution CT scan of the chest – This scan gives detailed pictures of the lungs and can show bronchiectasis, scars, and nodules that are common in long-term CVID lung disease.[65] [19]

20. Abdominal ultrasound or CT scan – Imaging of the belly can show enlarged liver or spleen, swollen lymph nodes, or signs of portal hypertension, which sometimes happen in CVID.[66] [20]

Non-Pharmacological Treatments (Therapies and Other Measures)

1. Infection-control education
Your care team teaches you simple things like washing hands often, using alcohol gel, and covering your mouth when you cough. These basic steps lower the number of viruses and bacteria you meet every day. For a person with CVID, fewer germs mean fewer infections and less lung damage over time. This education also includes knowing when to call the doctor early, so small infections do not become serious.

2. Avoiding crowded and high-risk places during outbreaks
During flu season or when there is a local outbreak (for example COVID-19 or RSV), your doctor may suggest avoiding packed buses, markets, or events. The purpose is to cut down your exposure to people who may be sick. The mechanism is simple: if you are in fewer close contacts, fewer germs reach your airways, and your risk of pneumonia or sinus infection goes down.

3. Vaccination of family and close contacts (cocooning)
You may not respond well to vaccines because of CVID, but your family and people who live with you usually can. Doctors often advise that your close contacts receive routine inactivated vaccines (like flu and COVID-19 shots). When the people around you are protected, germs are less likely to reach you. This “cocoon” effect protects you even if your own immune response is weak.

4. Avoiding live vaccines in most CVID patients
Most people with CVID should not receive live vaccines (such as oral polio, live nasal flu, or some travel vaccines), because these contain weakened but living germs. Your immune system may not control these safely. The purpose is to prevent vaccine-related infections. Doctors usually choose killed (inactivated) vaccines instead, when needed, and decide case by case.

5. Early treatment plan for infections
Many teams give you a written plan that explains what to do if you get fever, new cough, sinus pain, or diarrhea. Sometimes you keep “rescue” antibiotics at home, to start quickly after speaking with your doctor. The purpose is to treat infections early, before they spread to the lungs or blood. Early treatment reduces hospital stays and lung damage.

6. Airway-clearance techniques
If you already have bronchiectasis (damaged, widened airways), mucus can sit in your lungs and feed bacteria. Chest physiotherapy, huff coughing, and special breathing devices help move mucus up and out. The purpose is to clear thick secretions from the lungs, so germs cannot grow there easily. This lowers the risk of chest infections and protects lung function.

7. Pulmonary rehabilitation and breathing exercises
Some centers offer breathing exercises, supervised walking, and education for people with chronic lung disease. The aim is to improve lung capacity, reduce breathlessness, and build fitness safely. Better lung strength makes it easier for you to recover from infections and stay active in daily life.

8. Nasal saline rinses and sinus care
Daily saline sprays or rinses help wash out thick mucus, dust, and bacteria from your nose and sinuses. For people with CVID, chronic sinusitis is common, so keeping the nose clean can reduce pain, pressure, and repeated infections. The saline works by thinning mucus and helping tiny hairs in the nose move germs out.

9. Dental hygiene and regular dental visits
Mouth infections and gum disease can spread bacteria into the blood or sinuses. Brushing, flossing, and regular dental check-ups help control these problems. This lowers overall infection load in the body. It also helps prevent tooth loss and pain, which can make eating and nutrition more difficult.

10. Healthy, balanced nutrition
Your immune cells need protein, vitamins, and minerals to work as well as possible. A diet rich in fruits, vegetables, whole grains, lean protein, and healthy fats helps your body repair tissues and fight germs. Good nutrition also supports a healthy weight, which improves lung and heart function. Malnutrition makes infections more severe, so diet is a key part of care.

11. Regular physical activity
Gentle, regular exercise like walking, cycling, or swimming helps keep muscles and lungs strong. It also supports mental health and sleep. The mechanism is that exercise improves blood flow, which helps immune cells move around the body and reach places where they are needed. Your doctor can help you choose safe activities and avoid over-exertion when you are sick.

12. Smoking avoidance and clean-air habits
Smoking and second-hand smoke damage the lining of your airways and slow down the tiny hairs that move mucus out. For someone with CVID, this makes infections much more likely and worse. Avoiding smoking, vaping, and dusty or polluted workplaces helps protect your lungs. Air purifiers at home may also be helpful for some people.

13. Stress management and mental-health support
Living with a chronic disease can cause anxiety or low mood. Stress hormones can also affect how the immune system works. Talking therapy, support groups, relaxation exercises, and sometimes online counselling can help. The goal is to give you emotional tools so you can cope better and follow your treatment plan.

14. Patient education and support groups
Learning about CVID helps you notice warning signs early and work as a partner with your doctors. Many national immune-deficiency groups offer booklets, webinars, and peer support. The mechanism is empowerment: when you understand your condition, you can make safer choices and stick to therapies like Ig replacement and antibiotics.

15. Safe travel planning
Before travel, especially abroad, doctors may adjust your Ig dose, give travel letters, or prescribe standby antibiotics. You may also discuss vaccines that are safe for you. The purpose is to keep your infection risk low in new environments and far from your usual clinic. Planning also covers how to store Ig products and what to do in emergencies.

16. Home-based SCIG training
Many people with CVID learn to give immunoglobulin under the skin at home. Nurses teach sterile technique, pump use, and how to watch for side effects. This allows more flexible scheduling, less time in hospital, and more stable Ig levels. It also gives patients more independence and control over treatment.

17. Regular monitoring and screening tests
You will usually have regular blood tests, lung function tests, and sometimes CT scans or endoscopy. Doctors look for new problems like autoimmune disease, lung scarring, or cancers. The purpose is early detection, when problems are easier to treat and less likely to cause permanent damage.

18. Gastrointestinal care and dietitian support
Many people with CVID have chronic diarrhea, malabsorption, or weight loss. Seeing a gastroenterologist and a dietitian helps manage lactose intolerance, celiac-like disease, or bacterial overgrowth. Tailored diets and sometimes special formulas improve nutrient absorption, energy, and growth in children.

19. Infection-control at home (cleaning and food safety)
Simple home steps like safe food handling, cooking meat thoroughly, avoiding unpasteurized dairy, and regular cleaning of humidifiers reduce exposure to harmful germs. This helps prevent stomach infections and some respiratory infections. People with CVID benefit from these habits because they do not have a normal antibody “safety net.”

20. School and work accommodations
For children and adults, schools or employers can sometimes adjust schedules, allow extra sick days, or let you work from home during outbreaks. The purpose is to lower infection exposure while still supporting education and work. Good communication between you, your family, and your care team helps organize these changes.

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Drug Treatments

Important: Never start, stop, or change any medicine on your own. All drug treatments must be prescribed and adjusted by a specialist, especially in a young person.

1. Intravenous immunoglobulin (IVIG)
IVIG is pooled IgG antibodies from healthy donors, given into a vein every 3–4 weeks. It is the main treatment for CVID and is approved for primary immunodeficiency. Brands include Gammagard Liquid, Gamunex-C, Privigen, Octagam, and Flebogamma. Dose is based on body weight (often around 400–600 mg/kg every 3–4 weeks), adjusted to keep IgG at a safe level. It works by replacing the antibodies your body cannot make. Common side effects include headache, chills, and fatigue during or after infusion.

2. Subcutaneous immunoglobulin (SCIG)
SCIG is also IgG from donors but is given under the skin in small doses weekly or more often. Brands include Hizentra, Cuvitru, Xembify, and Cutaquig, which are cleared for subcutaneous use in primary immunodeficiency. SCIG gives more stable Ig levels and can often be done at home. Side effects are usually local, such as redness or swelling at the injection site.

3. HyQvia (IG + recombinant hyaluronidase)
HyQvia combines IVIG with recombinant human hyaluronidase, which helps the Ig spread under the skin so larger doses can be given less often (for example every 3–4 weeks). It is approved for adults with primary immunodeficiency. The purpose is to offer an option similar to IVIG but with the convenience of subcutaneous administration. Side effects can include local swelling, headache, and flu-like symptoms.

4. Prophylactic macrolide antibiotic (for example azithromycin)
Some people with repeated chest or sinus infections receive a low-dose macrolide antibiotic several times per week. The goal is to prevent infections before they start and to reduce lung damage. The dose and schedule are chosen by the doctor based on age, kidney function, and local resistance patterns. Possible side effects include stomach upset and, rarely, heart rhythm problems.

5. Other prophylactic oral antibiotics (for example amoxicillin)
In some cases, a daily or every-other-day penicillin-type antibiotic like amoxicillin is used to prevent sinus and ear infections. The mechanism is constant low-level killing of bacteria in the nose and throat, reducing the chance of infection flares. Side effects can include allergy, diarrhea, or yeast infections, so doctors weigh benefits and risks carefully.

6. High-dose antibiotics for acute infections
When a person with CVID develops pneumonia, severe sinusitis, or sepsis, doctors often use high-dose intravenous antibiotics in hospital. These may include broad-spectrum beta-lactams or other classes, chosen based on culture results and local guidelines. The purpose is to rapidly control serious infections and protect lungs and other organs. Side effects depend on the specific drug but can include allergy, kidney effects, or gut upset.

7. Inhaled bronchodilators (for example salbutamol/albuterol)
If CVID has led to asthma-like symptoms or chronic obstructive lung disease, doctors may prescribe inhaled bronchodilators. These relax the muscles around the airways, making breathing easier and helping clear mucus. They are taken with an inhaler or nebulizer when needed. Side effects can include tremor or a fast heartbeat in some people.

8. Inhaled corticosteroids
For people with airway inflammation, inhaled steroids may be used to reduce swelling inside the bronchi. This can help control cough and wheeze and may lower the number of flare-ups. They work by calming the immune reaction in the lungs. Side effects can include hoarse voice and oral thrush, so rinsing the mouth after use is important.

9. Systemic corticosteroids (for autoimmune complications)
Some CVID patients develop autoimmune problems such as low platelets or red blood cells. Short-term courses of oral or intravenous steroids like prednisone may be used to control these flares. Steroids lower inflammation and slow the immune attack on blood cells. Because long-term use has many side effects (weight gain, bone loss, infection risk), doctors aim for the lowest effective dose and shortest duration.

10. Rituximab
Rituximab is a monoclonal antibody that targets CD20 on B cells. In CVID, it is sometimes used for autoimmune cytopenias or interstitial lung disease when steroids alone are not enough. It works by reducing the number of B cells that may be driving autoimmune damage. It is given by intravenous infusion in cycles. Side effects include infusion reactions and higher risk of some infections, so close monitoring is needed.

11. Mycophenolate mofetil
Mycophenolate is an immunosuppressant that blocks DNA building in lymphocytes. It may be used in CVID patients with lung inflammation, autoimmune disease, or granulomatous disease that does not respond to steroids alone. It is taken by mouth. The goal is to control harmful inflammation while continuing Ig replacement to protect against infections. Side effects include stomach upset, low blood counts, and increased infection risk.

12. Azathioprine
Azathioprine is another oral immunosuppressant sometimes used for autoimmune or inflammatory complications of CVID. It reduces overactive immune cells and can help control chronic lung or gut inflammation. Doctors monitor blood counts and liver tests closely, because side effects include low white cells and liver irritation. It is usually combined with Ig replacement, not used alone.

13. Methotrexate (low-dose immunomodulatory use)
Low-dose methotrexate can be used in some autoimmune or rheumatologic complications linked to CVID, such as inflammatory arthritis. It calms down certain immune pathways. It is taken once weekly with folic acid to reduce side effects. Because it can affect the liver, bone marrow, and pregnancy, it is used only under specialist supervision with regular blood tests.

14. Proton-pump inhibitors (for severe reflux)
Strong stomach-acid blockers (like omeprazole) are sometimes used when reflux contributes to chronic cough, aspiration, or lung infections. By reducing acid, they may lower irritation in the esophagus and lungs. Side effects with long-term use can include low magnesium, bone loss, and gut infections, so doctors try to use the lowest effective dose.

15. Antifungal medicines
If a person with CVID develops repeated fungal infections (such as chronic thrush or lung fungal disease), medicines like fluconazole or other antifungals may be prescribed. These drugs kill or stop fungi from growing. They are tailored to the specific fungus and site of infection. Side effects may include liver test changes and drug interactions, so monitoring is needed.

16. Antiviral therapies
If chronic viral infections, such as herpes viruses, become a problem, doctors may use antivirals like acyclovir or valganciclovir. The purpose is to keep viral load low and prevent painful or dangerous flares. Dosing depends on kidney function and type of virus. Side effects include kidney effects and changes in blood counts, so blood tests are checked regularly.

17. Bone-protective medicines
Long-term steroids and chronic inflammation can weaken bones. Some patients need vitamin D, calcium, and sometimes bone-protective drugs like bisphosphonates. These help slow bone loss and reduce fracture risk. Doctors choose these medicines based on bone-density scans and overall risk.

18. Hepatology medicines for liver disease
CVID can cause liver problems such as nodular regenerative hyperplasia or autoimmune-like hepatitis. Depending on the pattern, doctors may use medicines like ursodeoxycholic acid or immunosuppressants to protect the liver. The goal is to slow scarring, control inflammation, and prevent liver failure. Side effects depend on the specific drug and need specialist monitoring.

19. Therapies for inflammatory bowel disease-like CVID enteropathy
Some people develop a gut disease that looks like celiac or inflammatory bowel disease. Treatments can include budesonide, mesalamine, or other IBD medicines. These calm gut inflammation and improve diarrhea and nutrient absorption. The exact choice and dose are guided by endoscopy and biopsy findings.

20. Hematopoietic stem-cell transplant (HSCT) medication regimens
In extremely severe and complicated cases, particularly in children, hematopoietic stem-cell transplantation may be considered. This includes many medicines: conditioning chemotherapy, antibiotics, antifungals, and immune-suppressive drugs to prevent rejection. HSCT is rare for CVID and is usually done only in expert centers after careful discussion of risks and benefits.

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Dietary Molecular Supplements

Evidence for supplements in CVID is limited. They may support general health but do not replace Ig therapy or medicines.

1. Vitamin D – Supports bone health and immune regulation; many people with chronic illness have low levels. Doctors often aim for a mid-normal blood level, using daily or weekly doses. Too much can harm kidneys, so blood tests are needed.

2. Vitamin A – Important for the lining of the gut and respiratory tract. In small, safe doses, it may help maintain barrier function, but high doses are toxic, especially for pregnancy and the liver. Any vitamin A supplement should be supervised and based on diet and blood levels.

3. Vitamin C – An antioxidant that helps white blood cells function and supports wound healing. Moderate daily doses from food and simple supplements are usually enough. Very high doses can cause stomach upset and kidney stones, so more is not always better.

4. Zinc – Needed for many immune enzymes. Mild zinc deficiency can weaken immune responses. Low-dose supplements may be used if diet is poor or blood levels are low. Too much zinc can cause nausea and reduce copper, leading to anemia or nerve problems, so dosing must be careful.

5. Selenium – A trace mineral with antioxidant roles in immune cells. Low selenium is linked to worse outcomes in some infections. Small supplements may be considered if intake is low, but high doses can cause hair loss and nail problems.

6. Folate (folic acid) – Important for making DNA in blood cells. Folate deficiency can worsen anemia or low white blood count. Supplements may be needed if gut absorption is poor or certain drugs (like methotrexate) are used, but must be balanced against vitamin B12 status.

7. Vitamin B12 – Also essential for blood cells and nerves. In CVID with gut involvement, B12 absorption may be reduced. Injections or high-dose tablets can restore levels and support energy and nerve function. Testing guides dose and schedule.

8. Omega-3 fatty acids (fish oil) – May reduce some inflammation and support heart health. For CVID, they do not replace any main treatment but can support general health when taken in moderate doses with food. High doses can increase bleeding risk, especially with blood-thinning medicines.

9. Probiotics (with caution) – Some doctors consider carefully selected probiotic strains to support gut health, but in immunodeficiency there is a small risk of probiotic bacteria entering the bloodstream. Any probiotic use must be discussed with your immunologist or gastroenterologist first.

10. High-protein nutritional supplements – When weight loss or low muscle mass is a problem, protein-rich drinks or powders may be added. Protein provides the building blocks for immune cells and healing. A dietitian can help choose safe formulas that do not worsen diarrhea or allergies.

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Regenerative, Immunity-Boosting, and Stem-Cell–Related Drugs

These are not routine for CVID and are usually considered only in very complex cases or research.

1. Hematopoietic stem-cell transplantation (HSCT) – Uses donor stem cells to rebuild the immune system. Conditioning chemotherapy and immunosuppressive drugs are used before and after the transplant. This is potentially “curative” for some primary immunodeficiencies, but for CVID it carries high risk and is reserved for selected severe cases in expert centers.

2. Experimental gene-modified stem-cell therapies
   For other immune defects like ADA-SCID, gene therapy using the patient’s own stem cells has shown benefit and has FDA-approved products. For CVID, similar approaches are still under study. These treatments involve collecting stem cells, correcting a gene in a lab, and giving them back after conditioning. They are only done in clinical trials.

3. Granulocyte colony-stimulating factor (G-CSF)
   If a CVID patient also has severe neutropenia (very low neutrophils), doctors may use G-CSF (like filgrastim) to temporarily boost white cell numbers. It acts on the bone marrow to release more neutrophils. Side effects can include bone pain and, rarely, spleen problems.

4. Thrombopoietin receptor agonists
   In severe immune thrombocytopenia linked to CVID, drugs that stimulate platelet production (like eltrombopag or romiplostim) may be used when other treatments fail. They increase platelet counts by acting on bone-marrow cells. These medicines have risks such as liver test changes and clotting, so specialist care is vital.

5. Biologic anti-cytokine drugs for specific inflammation
   In rare, severe inflammatory complications, doctors may use biologics that block TNF or other cytokines. These can calm overactive immune pathways but may increase infection risk. They are chosen only after careful review of biopsy and imaging results and always combined with Ig replacement.

6. Supportive hormone therapy for growth and puberty (in children)
   In children with growth delay due to chronic illness, endocrinologists may sometimes use growth hormone or other hormone therapies. These are not immune “boosters” but help support normal growth and development while main CVID treatment continues.

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Surgeries and Procedures

1. Functional endoscopic sinus surgery (FESS) – Used for severe, chronic sinusitis that does not improve with medicines. Surgeons open blocked sinus passages so mucus can drain and infections are less frequent.

2. Bronchoscopy and, rarely, lung surgery – Bronchoscopy lets doctors look inside the airways, remove mucus plugs, and take samples. In very damaged lung areas, segmental lung resection may sometimes be needed. The aim is to control infections and bleeding, and improve breathing.

3. Splenectomy (spleen removal) – In some patients with life-threatening autoimmune destruction of blood cells that does not respond to medicines, removing the spleen can help. Because the spleen also helps fight certain bacteria, patients will need vaccines and sometimes lifelong antibiotics afterwards.

4. Liver biopsy and related procedures – When liver tests are abnormal, a liver biopsy helps understand the type of disease. Sometimes interventional procedures are needed to manage portal hypertension or other complications. The purpose is to guide treatment and prevent liver failure.

5. Catheter or port placement for IV therapy – For people needing frequent IVIG or antibiotics, a long-term venous access device may be placed. This allows safer and easier repeated infusions but must be carefully cared for to avoid line infections.

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Prevention Tips

1. Take Ig replacement exactly as prescribed and do not miss infusions.

2. Treat infections early; call your doctor promptly for fever, new cough, chest pain, or severe diarrhea.

3. Keep good hand hygiene and avoid touching your face with unwashed hands.

4. Ask about prophylactic antibiotics or vaccines for your household if you have frequent infections.

5. Do not smoke or vape, and avoid smoky or very dusty environments.

6. Keep regular follow-up visits and routine tests with your immunologist, lung, gut, and liver doctors.

7. Maintain a healthy weight and balanced diet to support your body’s strength and healing.

8. Get enough sleep and manage stress, because tiredness and stress can worsen infections.

9. Plan travel in advance with your care team, including extra Ig and medicines.

10. Join patient support groups for tips, emotional support, and updated information.

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When to See a Doctor Urgently

You should seek medical care right away (emergency or urgent clinic) if you have:

• High fever (for example 38.5°C or higher) with chills or feeling very unwell.

• Shortness of breath, chest pain, or trouble breathing.

• Cough with blood, or very thick green or brown sputum.

• Severe headache with stiff neck or confusion.

• Severe abdominal pain, blood in stool, or nonstop vomiting.

• Very heavy bleeding, large bruises, or tiny red spots on the skin.

• Sudden yellow eyes or very dark urine.

For milder symptoms (earache, sinus pain, light cough, new diarrhea), call your immunologist or primary doctor the same day for advice. Never wait “to see if it goes away” when you have CVID and feel seriously sick.

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What to Eat and What to Avoid

1. Eat: plenty of colorful fruits and vegetables for vitamins and antioxidants. Avoid: skipping these and living on fast food only.

2. Eat: lean protein (fish, chicken, eggs, lentils, beans) to build immune cells and muscles. Avoid: very processed meats like sausages and salami most of the time.

3. Eat: whole grains (brown rice, oats, whole-grain bread). Avoid: constant large portions of sugary snacks, sweets, and sweet drinks.

4. Eat: safe dairy (pasteurized milk, yogurt, cheese) if you tolerate it. Avoid: unpasteurized milk, soft cheeses made from raw milk, and raw eggs.

5. Eat: healthy fats like olive oil, nuts, seeds, and avocado. Avoid: large amounts of deep-fried foods and trans-fat snacks.

6. Eat: fermented foods (yogurt with live cultures, if your doctor agrees) to support gut health. Avoid: self-starting strong probiotic pills without medical advice, because of infection risk in immunodeficiency.

7. Eat: small, frequent meals if you have gut problems, to keep energy up. Avoid: very large, fatty meals that worsen reflux or diarrhea.

8. Drink: clean water regularly to stay hydrated, especially with fever or diarrhea. Avoid: unsafe water, unfiltered well water, or drinks with a lot of caffeine or sugar.

9. Use: safe food-handling habits, like cooking meat fully and chilling leftovers quickly. Avoid: buffets or street foods kept warm for a long time, which can grow bacteria.

10. Follow: any special diet plan from your gastroenterologist or dietitian. Avoid: extreme diets (very low-carb, detox, or fad diets) that may cause nutrient lack.

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Frequently Asked Questions

1. Is CVID curable?
At present, CVID is usually a lifelong disease. Ig replacement and other treatments control symptoms and complications but do not “switch off” the underlying problem. Very rarely, stem-cell transplant may be considered, but it has serious risks and is not routine.

2. Will I need immunoglobulin treatment forever?
Most people with true CVID need lifelong Ig therapy to keep infections under control. If Ig is stopped, infections often return and can quickly damage lungs and other organs. Your doctor may adjust dose and schedule over time, but stopping completely is usually not advised.

3. Is SCIG better than IVIG?
Both SCIG and IVIG work well. SCIG gives more steady Ig levels and can often be done at home. IVIG uses less frequent, larger infusions, usually in clinic or day hospital. Your choice depends on lifestyle, side effects, and veins. Many studies show similar protection from infections with both routes.

4. Can I go to school or work normally?
Many people with CVID study and work. Ig therapy and infection-prevention steps reduce illness days. You may still need extra time off during infections or outbreaks. Talking with teachers or employers and your doctor can help arrange reasonable changes, like flexible hours or work-from-home days.

5. Can I play sports?
In most cases, yes. Gentle to moderate exercise is good for health. Your doctor may caution you about heavy contact sports if you have very low platelets, big spleen, or severe lung disease. Always ask before starting a new, intense activity.

6. Can I get pregnant if I have CVID?
Many people with CVID have successful pregnancies, with close monitoring. Ig doses may be adjusted, and there is careful watch for infections and autoimmune problems. This should be planned with your immunologist and obstetrician before conception if possible.

7. Will my children have CVID?
Some forms of CVID have genetic causes, but many cases are “complex” and not clearly inherited in a simple pattern. The risk to children is usually low but not zero. A clinical geneticist or immunologist can offer more detailed counselling based on your history and any gene testing.

8. Is it safe to have pets?
Most people with CVID can safely keep pets if they use good hygiene: hand-washing after touching animals, keeping litter boxes and cages clean, and avoiding pet bites or scratches. Reptiles and some exotic pets carry more risky germs, so discuss these with your doctor.

9. Can I drink alcohol?
Small amounts may be allowed in adults, but liver involvement is common in CVID. Alcohol can add extra strain to the liver and interact with medicines. Always ask your doctor what is safe for you, and avoid binge drinking.

10. Are “immune booster” products helpful?
Most over-the-counter immune boosters have little strong scientific proof, and some may even be unsafe in immunodeficiency. They do not replace Ig therapy. Never start herbal or high-dose vitamin products without checking with your immunologist.

11. Will I live a normal lifespan?
Outcomes vary. People diagnosed early, with good access to Ig and regular follow-up, often live many years and lead active lives. Severe lung disease, liver disease, or cancers can shorten life. This is why early diagnosis, consistent treatment, and screening for complications are so important.

12. Why do I still get infections even with Ig?
Ig therapy lowers infection number and severity but does not make risk zero. Some germs are not fully covered by donor antibodies, and structural damage (like bronchiectasis) makes infections easier. Dose adjustments, prophylactic antibiotics, and airway-clearance therapy can help.

13. Can CVID cause cancer?
People with CVID have a higher risk of some cancers, especially lymphoma and stomach cancer. This does not mean everyone will get cancer, but it is another reason for regular checks, prompt evaluation of new symptoms, and healthy lifestyle choices.

14. Does stress make CVID worse?
Stress itself does not cause CVID, but it can make coping harder and may affect sleep and habits like eating, exercise, and taking medicines. This can indirectly increase infection risk. Stress-management techniques and mental-health support are part of good care.

15. Who should be on my care team?
Ideally, your team includes an immunologist, primary-care doctor, nurse experienced in Ig therapy, and, when needed, specialists in lungs, gut, liver, and mental health. Working together, they can make a long-term, personalized plan that fits your age, lifestyle, and goals.

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: February 26, 2025.