Two Cases of Adult Onset Still's Disease with Concomitant Hemophagocytic Syndrome / 대한류마티스학회지

ABSTRACT

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.