Introduction
While arthritis and CVID are two separate medical conditions, they can sometimes occur together. In some cases, individuals with CVID may experience joint pain and inflammation, which can lead to arthritis. This is thought to be due to the immune system’s dysfunction in CVID, which can cause chronic inflammation throughout the body, including the joints.
What Is a Common Variable Immune Deficiency?
Common Variable Immune Deficiency (CVID) is the dysfunction of the immune system typically caused due to a mutation in a gene or genes. It can cause recurrent bacterial and rarely viral infections and is characterized by low protective antibodies (immunoglobulins) and a higher risk of infections. People who have a common variable immune deficiency cannot make antibodies against agents that can cause infection.
In the vast majority of common variable immune deficiency cases, the causes are unknown. But it has been identified that genetic mutation is the cause of 10 % of people with CVID. Therefore it is believed to occur due to the combination of both environmental and genetic factors. But the ecological factors are unclear.
Common symptoms of common variable immunodeficiency are:
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Fever.
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Weight loss.
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Nausea or vomiting.
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Runny nose.
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Food nutrient deficiencies.
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Swollen glands or lymph nodes.
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Cough with yellow, green, or brown phlegm or mucus.
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Wheezing or other breathing issues.
Common variable immunodeficiency is the most commonly occurring primary immunodeficiency in adulthood. It presents a wide range of clinical manifestations that often include non-infectious complications and heightened susceptibility to infections.
These versatile manifestations can make it difficult to differentiate between other infections resulting in a diagnostic delay and undertreatment with increased mortality and morbidity. The autoimmune reaction occurs in about 30 % of CVID patients, and it is an emerging cause of morbidity and mortality in these types of patients. CVID patients have a higher risk of getting autoimmune diseases. The treatment for CVID includes Ig (immunoglobulin) replacement therapy. It can help to stop the cycle of recurrent infections.
What Are the Possible Complications of CVID?
To avoid complications from CVID, they must be managed promptly and correctly as they may lead to the most severe conditions. CVID affects anyone above the age of 2 years, but it is often diagnosed only in their 30s or 40s. Since it is diagnosed at a later stage, there are chances that the complications might have been developed already due to recurrent infections.
The following are the possible complications of CVID:
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Bronchiectasis: Bronchiectasis, which is an obstructive lung disease, can be caused by recurrent lung infections or pneumonia. This can cause a permanent widening of the bronchi and the bronchioles in the lungs, which could complicate the treatment for CVID.
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Cancers: People with CVID have a higher risk of developing cancer, especially lymphoma. Cancer risk, including those involving the immune system cells, called non-Hodgkin lymphoma, stomach, and other gastrointestinal regions, is also commonly seen in people with CVID.
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Poor Response to Vaccines: Since the immune system is impaired in people with CVID, some vaccines cannot develop adequate immunity. This will make them unable to create the immunoglobulins required for long-lasting immunity. Therefore, some live vaccines are contraindicated in people with this condition. But still, since the T-cell response is often normal, some immunity can still be gained from vaccines.
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Granulomas: Lung infections can lead to granulomas, which occur in 10 % to 20 % of cases. In CVID, granulomas (lumps of inflamed tissues) are typically seen in the lungs or lymph nodes but rarely in the skin, spleen, brain, bone marrow, gastrointestinal tract, and kidney.
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Life Expectancy: Some particular CVID complications can be associated with a lower life expectancy.
What Is Arthritis?
Pain and swelling in one or more joints can occur due to arthritis. Stiffness and joint pain is the most common symptom of arthritis, which can worsen with age. Arthritis is not a single disease, but it is an umbrella term for multiple conditions that affect the joints in the whole body. Arthritis can occur gradually or suddenly. The other symptoms of arthritis may include redness, warmth, swelling, and decreased range of motion in the affected joints.
What Is Arthritis with CVID?
Arthritis is a life-threatening complication in common variable immunodeficiency (CVID) patients. The severity of symptoms can vary among people with CVID. CVID can increase the risk of getting digestive disorders, autoimmune disorders, blood disorders, and cancer in an individual. It can be inherited, or a person can develop it during their lifetime.
About 1 to 10% of people with CVID will have joint symptoms like rheumatoid arthritis. The symptoms of arthritis with CVID may include pain, joint swelling, stiffness, and deformity. In some instances, arthritis can be due to an infection of the joints or unrelated to the autoimmune process.
Immune disorders in CVID patients are rheumatoid arthritis, Sjogren's syndrome (a chronic autoimmune disease that affects the salivary glands and the tear glands), and systemic lupus erythematosus (an autoimmune disease that includes skin rash, erosion of joints, or even kidney failure).
Children and young adults with this arthritis-associated CVID syndrome typically present recurrent infections. Patients with polyarthralgia (pain in several joints in the body) and fever for several months might develop recurrent respiratory and gastrointestinal tract infections and pernicious anemia. These patients also have a higher risk of autoimmune diseases.
How to Diagnose Arthritis with CVID?
Arthritis with CVID can be diagnosed by performing immunological studies. These tests will show decreased levels of IgM (immunoglobulin M), IgG (immunoglobulin G), complete IgA (immunoglobulin A) deficiency, increased percentage of CD8 lymphocytes, and a reduced ratio of CD4:CD8 (ratio of T-helper cells and killer cells).
How to Treat Arthritis with CVID?
To treat arthritis with CVID, sufficient administration of the following agents is helpful as it results in the remission of arthritis.
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Intravenous immunoglobulin substitution.
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Antibiotics.
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Anti-inflammatory drugs.
A few months later, if hand radiograms are performed in patients with arthritis-associated CVID, it can show narrowing of the intra-articular space (space inside a joint between two bones) in the right radiocarpal metacarpophalangeal joints (between the metacarpal bones and the proximal phalanges of the fingers) along with multiple bone cysts and erosions. These erosions present are indicative of rheumatoid arthritis. Arthritis can present primary immunodeficiency in adults, especially with recurrent infections or autoimmune diseases. These patients require more advanced diagnostic procedures and the therapeutic cooperation of different specialists.
Conclusion
Despite the challenges of managing arthritis in individuals with CVID, with proper care and management, the affected individuals can lead fulfilling lives with minimal impact on their quality of life.
