싸이클로스포린으로 치료한 파종성 혈관내 응고증과 다발성 장기침범으로 발현한 성인형 스틸병 2예 = Two Cases of Adult Onset Still`s Disease with Disseminated Intravascular Coagulation and Multiple Organ Involvement Treated with Cyclosporine

다국어 초록 (Multilingual Abstract)

Adult onset Still`s disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis. It is characterized by spiking fever, evanescent skin rash, arthralgia or arthritis, hepatosplenomegaly and laboratory abnormalities including neutrophilic leukocytosis, abnormal liver function tests and raised levels of serum ferritin. Coagulation abnormality is a rare presenting feature but it might be life threatening when associated with hepatopathy and hematologic abnormalities. We report two cases of AOSD with disseminated intravascular coagulation and multiple organ involvement, which improved with glucocorticoid and cyclosporine combination therapy.

참고문헌 (Reference)

1 Yamaguchi M, "et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992" Tsunematsu T 424-30,

2 Omagari K, "Successful treatment with cyclosporin in adult-onset Still disease manifesting as acute hepatitis with marked hyperferritinemia" 326 : 148-151, 2003

3 "Retrospective monocentric study of 17 patients with adult Still's disease, with special focus on liver abnormalities" 50 : 192-195, 2003

4 Fautrel B, "Proposal for a new set of classification criteria for adult-onset still disease" 81 : 194-200, 2002

5 Kirino Y, "Increased serum HO-1 hemophaocytic syndrome and adult onset Still's disease; use in the differential diagnosis of hyperferritinemia" 7 : R616-R624, 2005

6 "Goebel FD. Cerebral involvement of adult onset Still's disease. Clin Rheumatol 1992" 276-9,

7 "Gerster JC. Still's disease in adults with disseminated intravascular coagulation. Schweiz Rundsch Med Prax 1991" 376-8,

8 Efthimiou P, "Diagnosis and management of adult onset Still's disease" 65 : 564-572, 2006

9 Ramirez C, "Clinical significance of increased serum ferritin levels" 122 : 532-534, 2004

10 Park JH, "Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporin A" 19 : 137-141, 2004

11 "Adult-onset Still's disease" 18 : 663-676, 2004

12 장영광, "Adult onset Still's disease에서 파종성 혈관내 응고와 다발성 장기부전이 동반된 1예" 5 : 262-268, 1998

13 Reginato AJ, "Adult onset Still's disease: experience in 23 patients and literature review with emphasis on organ failure" 39-57,

14 Masson C, "Adult Still's disease. Part II. Management, outcome, and prognostic factors" 62 : 758-765, 1995