It is simpler to say what an auto inflammatory disease isn't: it is not one of the more well-defined auto immune diseases such as rheumatoid arthritis or lupus. Auto inflammatory diseases are a newly defined and distinct group of illnesses that affect the body's immune system and cause inflammation.
The term 'auto inflammatory disease' was first coined in the 1990s after the discovery of the gene mutation responsible for an illness causing long-lasting fever and severe inflammation.
The body has an 'innate' immune system and an 'acquired (or 'adaptive') immune system. Auto inflammatory diseases differ from auto immune disorders because they arise from problems with the innate immune system. While the adaptive immune system uses antibodies to fight selected infections, the innate immune system fights anything and everything in the body that it sees as foreign. The innate immune system responds to attack with an immediate reaction that usually manifests itself as inflammation, swelling, or redness.
When both immune systems are working correctly, they build an effective barrier against disease. But sometimes the body attacks its own tissues by mistake. When this happens in the innate immune system, it is called auto inflammatory disorder. Symptoms include high fever, rash, and joint swelling. Auto inflammatory disorders also cause an increased risk of amyloidosis, a potentially deadly build-up of a blood protein in vital organs. Despite progress in understanding these distinct disorders, further research is needed to improve their diagnosis and treatment.
