Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease

被引:10
|
作者
Cimaz, Rolando [1 ]
Von Scheven, Annette [2 ,3 ]
Hofer, Michael [2 ,3 ]
机构
[1] AOU Meyer, I-50139 Florence, Italy
[2] CHU Vaudois, Lausanne, Switzerland
[3] HUG, Geneva, Switzerland
关键词
pediatric rheumatology; MACROPHAGE-ACTIVATION SYNDROME; RECEPTOR ANTAGONIST ANAKINRA; ADULT STILLS-DISEASE; RHEUMATOID-ARTHRITIS; IL-1; BLOCKADE; DOUBLE-BLIND; OPEN-LABEL; PHASE-II; INTERLEUKIN-18; EFFICACY;
D O I
10.4414/smw.2012.13582
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Systemic-onset juvenile idiopathic arthritis (SoJIA), sometimes called Still's disease, is a systemic inflammatory disease classified within the spectrum of juvenile idiopathic arthritis (JIA). It is an orphan disease with often a chronic course and a major impact on the affected children and their families. This disorder is unique in terms of clinical manifestations, prognosis and response to conventional immunosuppressants. The objectives of this review are to describe SoJIA and emphasise the recent advances in the pathogenesis and treatment, which have transformed the care and the prognosis of this potentially life-threatening paediatric condition.
引用
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页数:9
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