Two Cases of Refractory Adult-Onset Still's Disease Responding to Anakinra

치료 불응성 성인형 스틸병: 아나킨라 사용 후 임상 호전을 보인 증례 보고

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with variable clinical features. The interleukin (IL)-1 receptor antagonist anakinra has been proposed as an alternative effective treatment in refractory AOSD. We report, for the first time in Korea, two cases of refractory AOSD in which anakinra treatment produced a clinical response. The first patient had frequent clinical flare-ups with fever, sore throat, myalgia, and pleuritic chest pain despite treatment with methotrexate and etanercept. In the second patient, treatments with various immunosuppressive agents failed to control the disease activity. Treatment with anakinra 100 mg/day was initiated in both cases. A complete clinical remission and improvement in the laboratory parameters were observed. The steroid dose was tapered without further clinical flare-ups. Anakinra appears to be an effective alternative treatment modality in patients with AOSD refractory to conventional disease-modifying anti-rheumatic drugs and corticosteroid therapy.

성인형 스틸병은 전신적 염증 질환으로 기존의 치료에 반응하지 않는 경우가 드물지 않다. 인터루킨-1 길항제인 anakinra는 불응성 성인형 스틸병의 대체 치료로 제시되고 있다. 저자들은 아나킨라 치료로 임상 호전을 보인 두 명의 불응성 성인형 스틸병 환자를 경험하였기에 한국에서 처음으로 이를 보고하는 바이다.