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What is an Autoinflammatory Disease?

Autoinflammatory Diseases are disorders that involve the hyperactivation of the Innate Immune System. This causes inappropriate episodes of inflammation without an infection or injury being present. 

What is the Innate Immune System? 

 

The Innate Immune System is the body's first line of defense against foreign bodies, injuries, and pathogens. It quickly responds to infections and other danger signals. It is also considered the "non-specific" immune system because it responds the same way to all types of invasion. Activity from the innate immune response triggers the adaptive immune system. 

What is the difference between Autoinflammatory Disease and Autoimmune Disease? 

Autoinflammatory Diseases are disorders of the Innate Immune System whereas Autoimmune Diseases are disorders of the Adaptive Immune System.1
Autoinflammatory Diseases do not have the high antibody titers or the antigen-specific T-cells that are characteristic of Autoimmune Diseases. 2

What is the Adaptive Immune System? 

 

The Adaptive Immune System specifically targets the type of germ that is causing infection. While slower to react than the innate immune system, it is more accurate and remembers the germs so the next time it is faced, it can start fighting faster. This system is comprised of T-cells, B-cells, and Antibodies. When you have a dysregulation in this system causing the immune system to begin attacking healthy tissues, you get Autoimmune Disease.  3, 4

What are the similarities between Autoinflammatory and Autoimmune Diseases?

Both Autoinflammatory Diseases and Autoimmune Diseases involve immune system dysregulation and inflammation. They can be systemic (affecting multiple systems) or localized (organ-specific) inflammation. Symptoms can range from mild to severe in either and can be substantially debilitating. People regardless of ethnicity, age, or gender can be affected by these diseases. 
Some researchers believe that Autoimmune and Autoinflammatory Diseases could be a part of a spectrum of inflammatory disorders rather than two distinct groups. 5, 6

References

1

Jason An, Ashish Marwaha, Ronald M. Laxer 

Autoinflammatory Diseases: A Review

The Journal of Rheumatology Sep 2024, 51 (9) 848-861; DOI: 10.3899/jrheum.2023-1209

2

Shaw PJ, McDermott MF, Kanneganti T-D. Inflammasomes and autoimmunity. 

Trends Mol Med. 2011;17(2):57-64. doi:10.1016/j.molmed.2010.11.001 

3

InformedHealth.org [Internet]. Cologne, Germany: Institute for Quality and Efficiency in Health Care (IQWiG); 2006-. In brief: The innate and adaptive immune systems. [Updated 2023 Aug 14].  https://www.ncbi.nlm.nih.gov/books/NBK279396/

4

Cano RLE, Lopera HDE. Introduction to T and B lymphocytes. In: Anaya JM, Shoenfeld Y, Rojas-Villarraga A, et al., editors. Autoimmunity: From Bench to Bedside [Internet]. Bogota (Colombia): El Rosario University Press; 2013 Jul 18. Chapter 5.  https://www.ncbi.nlm.nih.gov/books/NBK459471/

5

El-Shebiny, E.M., Zahran, E.S., Shoeib, S.A. et al. Bridging autoinflammatory and autoimmune diseases. Egypt J Intern Med 33, 11 (2021). https://doi.org/10.1186/s43162-021-00040-5

6

Rosenbaum, J. Spondyloarthritis: Autoimmune or Autoinflammatory? Spondylitis Association of America.

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