Prieur A M
Unité d'Immuno-Hématologie pédiatriques, Hôpital des Enfants-Malades, Paris.
Presse Med. 2000 Mar 11;29(9):503-9.
FAVORABLE PROGNOSIS: The prognosis of systemic onset juvenile idiopathic arthritis varies. The most favorable outcome is observed when joint involvement is mild or delayed. No initial clinical or biological feature is indicative of severe prognosis except polyarticular involvement at onset. There is no HLA association. After 10 years follow-up, inflammatory features have disappeared in 50% of the cases, with some disability in 25%.
Some severe complications can occur during the course of systemic juvenile idiopathic arthritis: secondary amyloidosis (1 to 2%), spontaneous or drug-induced macrophage activation syndrome. Gold salts, sulfasalazine, or nonsteroidal antiinflammatory drugs may be implicated, associated or not with viral infection. Growth retardation is most often secondary to corticosteroid therapy. Long-term prognosis as published in various series mentions 8 to 14% mortality, secondary to iatrogenic accidents, infections or secondary amyloidosis.
预后良好：全身型幼年特发性关节炎的预后各不相同。当关节受累较轻或延迟出现时，观察到的预后最为良好。除了起病时多关节受累外，没有初始临床或生物学特征可提示预后严重。不存在HLA相关性。经过10年随访，50%的病例炎症特征消失，25%的病例存在一定残疾。
在全身型幼年特发性关节炎病程中可能会出现一些严重并发症：继发性淀粉样变性（1%至2%）、自发性或药物性巨噬细胞活化综合征。金盐、柳氮磺胺吡啶或非甾体抗炎药可能与之相关，无论是否伴有病毒感染。生长发育迟缓最常见于皮质类固醇治疗的继发情况。各种系列报道的长期预后提及死亡率为8%至14%，继发于医源性意外、感染或继发性淀粉样变性。
