1. National Institute of Arthritis and Musculoskeletal and Skin Diseases. "Understanding Autoinflammatory Diseases" 2016.


The immune system is the body’s natural defense mechanism: when a harmful substance (such as an infection) is present, an immune response attacks that foreign substance, thus keeping us healthy or helping us recover.  However, when the immune system is not working properly, such as in autoimmune or autoinflammatory disease, this immune response is directed at the body’s own tissues healthy cells instead. (Reference 1) This can cause symptoms ranging from mild to severe, possibly leading to organ failure and disability. 

The immune system is comprised of two parts: 


Innate immunity means that genetic mutations are the primary cause of the immune response.  Autoinflammatory diseases stem from the innate immune system.  They are characterized by episodes of apparently unprovoked inflammation (no specific environmental influence).  Since they tend to have a higher genetic proponent than autoimmune diseases, they typically have early onset, meaning that they often present in childhood or early adulthood.  These diseases are typically marked by rashes and spiking fevers.

Acquired or Adaptive  

Acquired (adaptive or specific) immunity is triggered by a learned response. As a person's immune system encounters foreign substances (antigens), the components of acquired immunity learn the best way to attack each antigen and begin to develop a memory for that antigen.  Autoimmune diseases are the result of a faulty acquired immune system.  They involve some combination of genetic and environmental triggers.  It is believed that the greater the genetic involvement, the earlier the disease will present; the later the onset, the higher likelihood there is a strong environmental trigger (gut or lung bacteria, smoking, etc.).

For more information about the differences between the different types of immune system malfunction, visit the Autoinflammatory Alliance.