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Home / Understanding Immune Deficiencies

Understanding Immune Deficiencies

Immune deficiencies are conditions where the immune system doesn’t work properly, making it harder for the body to fight infections and other diseases. Doctors and specialists around the world classify and manage these conditions based on specific gene defects and international guidelines, such as those from the International Union of Immunological Societies (IUIS) and the European Society for Immunodeficiencies (ESID).

Evolving Understanding of Immune Deficiencies

Over the past 10 to 15 years, our understanding of immune deficiencies has greatly improved, thanks to advanced techniques like exome and genome sequencing. These techniques help identify specific genetic errors that cause not only immune deficiencies but also autoimmune diseases, inflammatory conditions, bone marrow problems, and cancer predispositions.

From PID to IEI

Traditionally, these conditions were called primary immune deficiencies (PIDs). However, as our knowledge expanded, it became clear that the term PID didn’t fully capture the range of these disorders. In 2017, the IUIS introduced the term inborn errors of immunity (IEIs) to better reflect the diverse nature of these conditions. IEIs include not only deficiencies in immune function but also predispositions to immune system overreactions, bone marrow failures, and certain cancers.

Primary vs. Secondary Immune Disorders

An important distinction in the world of immune disorders is between primary (inborn or genetic) and secondary (acquired due to other causes) immune disorders:

  1. Primary Immune Disorders (PIDnew or IEIs): These are due to inherent defects in the immune system, whether present from birth or acquired later. They are often genetic and can involve a single gene or multiple genes.
  2. Secondary Immune Disorders (SIDnew): These occur due to other medical conditions, treatments, or environmental factors. For example, certain cancer therapies can weaken the immune system, leading to secondary immune deficiencies.

Why This Distinction Matters

Understanding whether an immune disorder is primary or secondary is crucial for treatment:

  • Primary Immune Disorders: Treatment focuses on correcting the specific immune defect, often with genetic therapies or other targeted treatments.
  • Secondary Immune Disorders: Treatment aims at addressing the underlying cause (like stopping a particular medication) and providing supportive care until the immune system recovers.

Importance of Accurate Classification

Accurate classification and understanding of these conditions are vital for effective treatment and ongoing research. The newer terms and classifications help doctors provide better care and guide the development of new therapies.

Moving Forward

There is a proposal to reintroduce the term PID (as PIDnew) to replace IEI, as it’s a familiar term that many find easier to use and understand. This change aims to improve communication in scientific and medical communities, as well as education for patients and their families.

Key Takeaways

  • Primary Immune Deficiencies (PIDs): Now often called inborn errors of immunity (IEIs), these include a wide range of genetic disorders affecting the immune system.
  • Secondary Immune Disorders (SIDs): These are caused by external factors like other illnesses or treatments.
  • Treatment and Management: Differ depending on whether the disorder is primary or secondary, with primary disorders often requiring more specific, targeted treatments.
  • Screen newborns for immune deficiencies: Just like HIV research has led to important advances in immunology, such as the T-cell receptor excision circle (TREC) test used to screen newborns for immune deficiencies, modern research techniques that study all the genes and proteins in a person (called “omics” research) are now important for accurately diagnosing and treating secondary immune disorders.

Understanding these terms and distinctions helps patients and their families better navigate the complexities of immune disorders and the available treatments.

Ref Journal of Allergy & Clinical Immunology



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