Primary Immunodeficiency disorders (PID) are a group of over 400 rare conditions that impair the immune system, making children vulnerable to recurrent infections, allergies, and other health complications. While managing infections is the immediate concern, children with PI are also at a higher risk of developing autoimmune diseases and certain cancers, adding to the complexity of their care.
Understanding Primary Immunodeficiency
While interacting with OnlyMyHealth team, Dr Preeti Anand, Senior Consultant, Paediatrics, Max Smart Super Speciality Hospital, Saket, New Delhi, explains, "Primary Immunodeficiency is a group of over 400 disorders that impair the immune system, making children susceptible to recurrent infections, allergies, and other health complications. While the immediate concerns often revolve around managing infections, research shows that children with PI are also at an elevated risk of developing autoimmune diseases and certain types of cancers."
Autoimmune Diseases and PID
One of the paradoxes of primary immunodeficiency is that while these disorders weaken the immune system, they can also cause it to turn against the body itself. In a healthy immune system, the body distinguishes between its own cells and foreign invaders. However, in children with PI, this ability can be compromised, leading to autoimmunity, where the immune system mistakenly attacks the body's own tissues.
Dr Preeti highlights, "The prevalence of autoimmune diseases in children with PI is notably higher than in the general population. Autoimmune conditions such as autoimmune hemolytic anemia, juvenile idiopathic arthritis, and systemic lupus erythematosus are more common among these children. The underlying mechanisms involve the dysregulation of immune tolerance, where the immune system fails to properly control autoreactive cells that target the body’s own tissues."
Cancer Risks
The increased risk of malignancies is another serious concern for children with PID. The immune system plays a critical role in identifying and destroying cancerous cells, but when this system is compromised, the body’s natural cancer surveillance is weakened. As a result, children with PI have a heightened risk of developing cancers, especially lymphomas and leukemias.
Studies have shown that the incidence of cancer in children with PI is significantly higher than in those without these disorders. Dr Preeti says, “Lymphomas, particularly non-Hodgkin lymphoma, are among the most common cancers seen in these children. The risk is further compounded by chronic viral infections, such as Epstein-Barr virus (EBV), which can trigger oncogenic processes in the absence of a fully functional immune system."
Chronic Inflammation and Additional Complications
Beyond autoimmunity and cancer, chronic inflammation is a persistent issue for children with PI. Ongoing infections and the immune system's continuous battle to fight them can lead to chronic inflammatory conditions, causing tissue damage and further health complications like inflammatory bowel disease (IBD) and interstitial lung disease.
Children with PID also face growth and developmental delays due to frequent infections and the long-term effects of intensive treatments. Regular hospitalisations, surgeries, and aggressive antibiotic therapies can significantly affect their quality of life.
Managing Risks and Improving Quality of Life
Early diagnosis and a tailored treatment plan are essential for managing the risks associated with primary immunodeficiency. Treatments such as immunoglobulin replacement therapy, antibiotics, and, in some cases, hematopoietic stem cell transplantation (HSCT) can help control infections and reduce complications. Close monitoring for signs of autoimmune diseases and cancers is critical for timely interventions.
Conclusion
While children with primary immunodeficiency face challenges beyond infections, advances in medical research and personalized treatment strategies provide hope. Dr Preeti Anand emphasizes the importance of a multidisciplinary approach involving immunologists, oncologists, rheumatologists, and other specialists to offer comprehensive care and improve the quality of life for these children.