식혈증후군이 동반된 성인형 스틸병은 드물지만 치명적인 결과를 가져올 수 있기 때문에 조기에 병을 진단하고 일찍 치료를 시작하는 것이 중요하며, 저자들은 골수 검사로 식혈 증후군이 확진된 성인형스틸병 2예를 경험하였기에 이에 문헌고찰과 함께 보고하는 바이다.

Adult onset Still`s disease is an rare inflammatory disease with the characteristic of fever, skin rash, arthralgia or arthritis, lymphadenopathy, leukocytosis and multiple systemic organ involvement. Its accurate pathogenesis has not been elucidated yet. Its clinical manifestation is also very diverse, from relatively mild symptoms to severe complications such as concomitant infection, liver failure, disseminated intravascular coagulation, myocarditis, adult respiratory distress syndrome, which may lead to death in some cases. Particularly, concomitant hemophagocytic syndrome is rare complication that could induce a fatal outcome. Thus it is important to diagnose early and start treatments. Until now, it has been reported in only one case of adult onset Still`s disease in Korea. Here, we report two female cases of adult onset Still`s disease with concomitant hemophagocytic syndrome.