Mouy R, Stephan J L, Pillet P, Haddad E, Hubert P, Prieur A M
Pediatric Rheumatology Unit, Hôpital des Enfants-Malades, Université Paris V, France.
J Pediatr. 1996 Nov;129(5):750-4. doi: 10.1016/s0022-3476(96)70160-9.
To evaluate the efficacy of cyclosporine A in the treatment of macrophage activation syndrome (MAS) occurring in children with juvenile arthritis.
MAS developed in two boys and three girls with systemic juvenile arthritis (four) and polyarticular juvenile arthritis (one). In three children whose condition was life-threatening, increased parenteral administration of corticosteroids failed to improve their condition; therefore cyclosporine A (2 to 5 mg/kg per day) was added. In two other patients with less severe clinical manifestations, cyclosporine A alone (2 to 8 mg/kg per day) was given.
After the introduction of cyclosporine A, rapid improvement was obtained in all patients and apyrexia occurred within 24 to 48 hours. The biologic abnormalities disappeared more slowly (up to 5 weeks for liver enzymes).
These observations underline the usefulness of cyclosporine A in this complication. The use of this drug may circumvent the need for increased doses of corticosteroids in some patients. The mechanism of action of cyclosporine A remains speculative, but these results indicate indirectly that T-helper lymphocytes may play a role in the pathogenesis of MAS.
评估环孢素A治疗幼年特发性关节炎患儿巨噬细胞活化综合征（MAS）的疗效。
两名男孩和三名女孩发生了MAS，其中四名患有全身型幼年特发性关节炎，一名患有多关节型幼年特发性关节炎。在三名病情危及生命的儿童中，增加肠外给予皮质类固醇未能改善病情；因此加用了环孢素A（每日2至5毫克/千克）。另外两名临床表现较轻的患者单独给予环孢素A（每日2至8毫克/千克）。
加用环孢素A后，所有患者均迅速好转，发热在24至48小时内消退。生物学异常消失得较慢（肝酶异常长达5周才消失）。
这些观察结果强调了环孢素A在这种并发症治疗中的作用。使用该药可能避免一些患者需要增加皮质类固醇剂量。环孢素A的作用机制仍属推测，但这些结果间接表明辅助性T淋巴细胞可能在MAS的发病机制中起作用。
