Abstract
The autoinflammatory diseases are Mendelian-inherited monogenic diseases that cause systemicinflammation. Most of the responsible genes for the autoinflammatory diseases areinvolved with the innate immunity, especially pattern recognition receptors. Dr. Kastner inNational Institutes of Health has introduced the term “autoinflammatory diseases”for the firsttime in 1999 in the report that identifies the responsible gene for TRAPS, so the field of the autoinflammatory diseases is relatively new. Since the patients with the autoinflammatorydiseases suffer from fever of unknown origin, arthritis, arthralgia, and rash, rheumatologists haverelatively higher probabilities to see these patients. Although their prevalence are very low, appropriate therapies will change QOL of these patients so dramatically that the rheumatologistsshould know the clinical features of the autoinflammatory diseases. In Japan, the nationwidesurveys on the autoinflammatory diseases had been conducted from 2009, which helped us tounderstand the pictures of autoinflammatory diseases in Japan. In this review, I am going todescribe what we have learned from the surveys and what needs to be done in the field ofautoinflammatory diseases.
