Abstract
Autoinflammatory diseases have recently been recognized as diseases characterized by systemic inflammation mediated by abnormalities in the molecules of the innate immune system. Clinical features of the systemic onset juvenile idiopathic arthritis (sJIA), the most common rheumatic disease in Japan, mimic those of autoinflammatory disease. In addition, a large number of evidence indicates the pathogenesis of sJIA to be closely associated with abnormalities in the innate immune system rather than with the classic autoimmune system. Based on these findings, a consensus is now emerging that sJIA may be a autoinflammatory disease. In order to deepen our understanding of human innate immunity, and to offer more targeted therapies for patients with sJIA, further studies on the genetics and molecular pathophysiology of sJIA and autoinflammatory diseases are essential.
