혈전성 혈소판감소성 자반증이 동반된 성인형 스틸병
Adult-Onset Still’s Disease with Thrombotic Thrombocytopenic Purpura
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
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.
Abstract
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.
참고문헌 (10)
- Yamaguchi M, "Preliminary criteria for classification of adult Still’s disease", J Rheumatol, 19, pp.424~430 (1992)
- 김현숙, "식혈증후군이 동반된 성인형 스틸병 2예", 대한류마티스학회지, 14(2), pp.160~165 (2007), 대한류마티스학회
- George JN, "Syndromes of thrombotic microangiopathy", N Engl J Med, 371, pp.654~666 (2014)
- Masuyama A, "case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema", Mod Rheumatol, 23, pp.379~385 (2013)
- Furlan M, "von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome", N Engl J Med, 339, pp.1578~1584 (1998)
- Rieger M, "ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases", Blood, 106, pp.1262~1267 (2005)
- Mannucci PM, "Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis)", Haematologica, 88, pp.914~918 (2003)
- Zbaras B, "Thrombotic thrombocytopenic purpura associated with adalimumab (Humira) treatment in Crohn disease", Intern Med J, 43, pp.216~217 (2013)
- Bessissow T, "Review article: non-malignant haematological complications of anti-tumour necrosis factor alpha therapy", Aliment Pharmacol Ther, 36, pp.312~323 (2012)
- Sumida K, "Etanercept-refractory adult-onset Still’s disease with thrombotic thrombocytopenic purpura successfully treated with tocilizumab", Clin Rheumatol, 29, pp.1191~1194 (2010)