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Case Report
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Korean J Med. 2014;87(2):245-250. Published online August 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.87.2.245
- 아나킨라로 호전된 급성 폐렴을 동반한 성인형 스틸병
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조성하, 김원중, 조상우, 임동민, 안수경, 김춘관
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중앙보훈병원 내과
- A Case of Adult Onset Still’s Disease with Severe Pneumonitis Treated with Anakinra
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Soung Ha Cho , One Zoong Kim , Sang Woo Cho , Dong Min Lim , Su Kyoung An , Choon Kwan Kim
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Department of Internal Medicine, VHC Medical Center, Seoul, Korea
- Corresponding author: Choon Kwan Kim ,Tel: +82-2-2225-1491, Fax: +82-2-2225-4374, Email: schenics@naver.com
- Received: September 11, 2013; Revised: October 16, 2013 Accepted: November 26, 2013.
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߽ɾ :성인형 스틸병; 폐렴; 아나킨라
- Abstract
- Adult onset Still’s disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still’s disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient’s disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra.
Keywords :Adult onset Still’s disease, Pneumonitis, Anakinra
