Autoinflammatory disease

For acquired immunodeficiency syndrome (AIDS), see HIV/AIDS.

Not to be confused with Autoimmune disease.

Autoinflammatory diseases (AIDs) are a group of rare disorders caused by dysfunction of the innate immune system. These responses are characterized by periodic or chronic systemic inflammation, usually without the involvement of adaptive immunity.

Autoinflammatory diseases are a separate class from autoimmune diseases; however, both are characterized by an immune system malfunction that may cause similar symptoms, such as rash, swelling or fatigue. However, the main source of the diseases are different. A key difference between the two classes of diseases is that while autoinflammatory disease triggers a malfunction of the innate immune system, autoimmune diseases trigger a malfunction of the adaptive immune system.

The distinction between autoinflammatory (overactivity of the innate immunity), autoimmune (overactivity of the adaptive immunity) and immunodeficient (decreased activity of the innate or adaptive immunity) is not concretely defined. Clinical phenotypes associated with autoinflammatory conditions are due to the type of cell vulnerable to cellular processes, often due to a specific mutation or cellular signal which may present as excessive activation of neutrophils, monocytes/macrophages and dendritic cells causing auto-inflammatory symptoms, or T cell and B cell dysfunction leading to autoimmunity. Failure of innate and/or adaptive immune cells to appropriately activate, recognize, and clear infectious agents causes immunodeficiency and vulnerability to infection.