Lymphoma refers to cancer that originates in the lymphatic system, which is part of the body’s immune system. Lymphoma develops when lymphocytes, a type of white blood cell, undergo changes and start growing uncontrollably. There are many different types of lymphoma, but some forms can be caused by autoimmune diseases.
What is an autoimmune disease?
An autoimmune disease occurs when the immune system mistakenly attacks healthy cells in the body. Normally, the immune system is able to tell the difference between foreign invaders like bacteria and viruses, and the body’s own healthy cells. In autoimmune diseases, that ability to distinguish breaks down. The immune system begins producing antibodies that target the body’s own tissues and organs.
Some common autoimmune diseases include rheumatoid arthritis, lupus, type 1 diabetes, inflammatory bowel disease, multiple sclerosis, and more. These diseases can damage various parts of the body such as joints, skin, organs, connective tissues, and blood vessels. Treatment for autoimmune diseases focuses on managing symptoms and preventing further damage.
The link between autoimmunity and lymphoma
Research shows that having certain autoimmune diseases increases the risk of developing lymphoma. Some types of autoimmune conditions are more closely associated with lymphoma than others. The reasons for the connection are not fully understood, but some possible explanations include:
- Chronic immune system stimulation – Autoimmune diseases cause persistent activation of immune cells like lymphocytes to attack healthy tissue. This constant stimulation may encourage cancerous growth.
- Inflammation – Autoimmune diseases generate inflammation in tissues and organs. Chronic inflammation can produce DNA damage and promote tumor growth.
- Treatment side effects – Some medications used to treat autoimmune diseases may increase cancer risk. For example, immunosuppressants restrain the immune system, potentially enabling abnormal cell growth.
Genetic factors may also play a role in the relationship between autoimmunity and lymphoma development in susceptible individuals.
Major autoimmune diseases connected to lymphoma
The autoimmune conditions with the strongest associations with lymphoma include:
Rheumatoid Arthritis
Rheumatoid arthritis (RA) causes joint inflammation, pain and damage throughout the body. It occurs when the immune system mistakenly attacks the lining of the joints. RA patients have a higher risk of developing certain lymphomas, particularly diffuse large B-cell lymphoma. The risk seems greatest for those with highly active, severe rheumatoid arthritis.
Sjögren’s Syndrome
Sjögren’s syndrome damages moisture-producing glands leading to dry eyes and dry mouth. Sometimes other organs are affected. It is caused by abnormal immune reactions. Sjögren’s syndrome patients have a nearly 40 times greater risk of developing lymphoma compared to the general population. The lymphomas are usually of B-cell origin.
Celiac Disease
Celiac disease is an immune reaction to gluten, a protein found in wheat, barley and rye. Eating gluten triggers inflammation and damage in the small intestine. Celiac disease boosts the possibility of lymphoma, particularly enteropathy-associated T-cell lymphoma of the small intestine. Strictly following a gluten-free diet may help lower lymphoma risk.
Autoimmune Thyroiditis
Also called Hashimoto’s thyroiditis, this is the most common cause of hypothyroidism. The immune system attacks and destroys the thyroid gland. There is a link between chronic thyroiditis and development of malignant lymphoma, but the association is not fully proven.
Psoriasis
Psoriasis causes red, itchy, scaly patches on the skin. It results from overactive immune responses. Psoriasis is connected to an increased chance of lymphoma, particularly cutaneous T-cell lymphoma. Patients with severe psoriasis that started early in life seem to have the highest risk.
Inflammatory Bowel Disease
Inflammatory bowel diseases like Crohn’s disease and ulcerative colitis involve chronic inflammation of the digestive tract. Studies show patients with long-standing inflammatory bowel disease have a higher rate of lymphoma. Treatment with the drug infliximab may further increase lymphoma risk.
Diagnosing autoimmune-associated lymphoma
When lymphoma develops in the setting of an autoimmune disease, patients often initially present with symptoms related to a flare-up of their chronic condition. For example, worsening joint pains and swelling could signal rheumatoid arthritis-associated lymphoma. Gradually worsening fatigue, appetite changes and weight loss could indicate lymphoma in celiac disease.
Doctors may order imaging tests like CT scans or PET scans if lymphoma is suspected. A lymph node biopsy is necessary to confirm a diagnosis of lymphoma. This involves surgically removing an affected lymph node or part of a node for examination under a microscope.
Immunophenotyping and other specialized tests help identify specific lymphoma subtypes. Determining the type of lymphoma is necessary to select the most appropriate treatment.
Treating autoimmune-related lymphoma
Treatment depends on the individual case, including the type and stage of lymphoma, as well as the severity of the underlying autoimmune disease. In early stage lymphomas, radiation therapy aimed at affected lymph nodes may be sufficient. More advanced disease requires chemotherapy, usually combined with targeted drugs like rituximab.
For some autoimmune diseases associated with very high lymphoma risk, doctors may recommend lower doses of immunosuppressant medications to avoid excess immune system impairment. Close monitoring with frequent medical exams is also important.
In addition to treating the lymphoma, managing the autoimmune disease is also key. This may require adjusting doses of medications like steroids, DMARDs and biologics to balance control of autoimmunity with avoiding over-suppression of the immune system.
Preventing autoimmune-related lymphoma
It can be difficult to prevent lymphoma that stems from autoimmune dysfunction, but some strategies may help lower risk:
- Gain control over autoimmune disease activity – Keeping chronic immune responses in check may reduce lymphomagenesis.
- Use biologics cautiously – Newer biologic drugs powerfully suppress inflammation but possibly increase lymphoma risk.
- Limit doses/duration of immunosuppressants when possible.
- Treat infections promptly – Chronic infections promote immune stimulation.
- Follow healthy lifestyle practices – Exercise, nutrition, sleep hygiene, stress management.
- Screen for lymphoma – Monitor for lump/swelling, get frequent medical exams.
Awareness of the link between autoimmunity and lymphoma prompts earlier detection and facilitates better outcomes. Patients should stay vigilant for new symptoms and discuss any concerns promptly with their healthcare team.
Conclusion
Several autoimmune diseases including rheumatoid arthritis, Sjögren’s syndrome, celiac disease and others confer an increased risk of developing certain types of lymphoma. The constant immune activation and inflammation generated by autoimmunity appears to fuel lymphomagenesis in genetically predisposed patients. There are strategies to potentially reduce, but not eliminate, the risk of lymphoma. Treatment focuses on controlling both the autoimmune condition and the blood cancer for optimal outcomes.