혈전성 혈소판감소성 자반증이 동반된 성인형 스틸병 (Adult-Onset Still’s Disease with Thrombotic Thrombocytopenic Purpura)

서지정보

· 발행기관 : 대한내과학회
· 수록지 정보 : 대한내과학회지 / 89권 / 4호 / 482 ~ 486페이지
· 저자명 : 이태한, 이지민, 이소연, 조난희, 손창남, 김지민, 김상현

초록

성인형 스틸병은 다양한 임상증상을 보이는 드문 질환으로TTP가 동반되는 경우는 매우 드물고 사망률 또한 높다. 이에 저자들은 스테로이드, 항TNF-α제제, 면역 글로불린, 혈장 교환술 치료에 반응이 없었던 TTP를 동반한 성인형 스틸병을경험하였기에 국내 최초로 보고하는 바이다.

영어초록

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder that affects multiple organs and is fatal in some cases. Thrombotic thrombocytopenic purpura (TTP), another rare disease, is characterized by multiple organ failure and microangiopathic hemolytic anemia. TTP is an extremely rare complication of AOSD. We report a 59-year-old woman who presented with TTP that manifested after she was diagnosed with AOSD. Initially, her clinical manifestations improved with glucocorticoid therapy. However, her disease worsened and was accompanied by acute kidney injury, thrombocytopenia, hemolytic anemia, petechiae, and impaired consciousness. These clinical findings led to a diagnosis of TTP. This is the first report of AOSD complicated by TTP in Korea. Awareness of the possible development of TTP as a complication of AOSD is important for early diagnosis and treatment.

참고자료

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