중증 대식세포활성증후군이 합병된 소아 특발성 관절염 환자에서 발생한 중추성 갑상선기능저하증 1예

A case of central hypothyroidism developed in male child with severe macrophage activation syndrome complicated by juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is chronic inflammatory arthritis of unknown origin, and it is believed that genetic and environmental factors are involved. Macrophage activation syndrome (MAS) is complication of JIA, which occurs in 5-8% of systemic JIA and can be fatal due to fever, central nervous system disorder and hematologic disorder. JIA is thought to be involved in autoimmune reaction and is actually associated with other autoimmune disease such as autoimmune hypothyroidism. However, association with other thyroid diseases has not been found. We report a case of central hypothyroidism developed in MAS as a complication of JIA patient, who was 2 year 3 month old boy. He was treated by HLH 2004 regimen including combination of etoposide, cyclosporine A, dexamethasone.