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首页> 外文期刊>Journal of clinical rheumatology >Treatment of pediatric Takayasu arteritis with infliximab and cyclophosphamide: Experience from an American-Brazilian cohort study
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Treatment of pediatric Takayasu arteritis with infliximab and cyclophosphamide: Experience from an American-Brazilian cohort study

机译：英夫利昔单抗和环磷酰胺治疗小儿高寒动脉炎：美国-巴西队列研究的经验

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Background: Pediatric Takayasu arteritis (pTA) is difficult to treat and can lead to significant morbidity and mortality. Objectives: The objective of this study was to describe clinical characteristics for pTA and determine the safety and efficacy of cyclophosphamide (CYC) and infliximab (IFX) in pTA. Methods: This was a retrospective analysis of 23 pTA patients seen at Children's Hospital Los Angeles and Universidade Federal de S?o Paulo-Brazil from 1990 to 2011. All patients fulfilled the 1990 American College of Rheumatology criteria for Takayasu arteritis. Disease activity was assessed using a modified National Institutes of Health score. Results: Twenty-three patients (14 female and 9 male patients), mean age of 15.7 ± 6.0 years, were included. Cyclophosphamide was used before IFX treatment in 17 of 23 and IFX before CYC in 2 of 23 patients. The average time from disease onset to treatment initiation was 2.6 ± 2.4 years for CYC and 4.1 ± 2.4 years for IFX. Nine (60%) of 15 patients failed CYC, and of these 6 were changed to IFX with subsequent clinical stabilization in 5 (83%) of 6. Two patients initially treated with IFX were switched to CYC because of lack of appropriate response: 1 patient later worsened, and the other was lost to follow-up. Five opportunistic infections requiring hospitalization occurred in the CYC group, whereas none were observed in the IFX group. Patients in the IFX group were more likely to decrease or stop their corticosteroids when compared with the CYC patients. Conclusions: Cyclophosphamide is often used as initial treatment but has many adverse effects. In this retrospective case series, IFX was equivalent to CYC with fewer adverse effects, making IFX an alternative therapeutic option for pTA.
机译：背景：小儿高筋动脉炎（pTA）难以治疗，可导致明显的发病率和死亡率。目的：本研究的目的是描述pTA的临床特征，并确定pTA中环磷酰胺（CYC）和英夫利昔单抗（IFX）的安全性和有效性。方法：这是一项回顾性分析，分析了1990年至2011年在洛杉矶儿童医院和联邦圣保罗巴西大学的23例pTA患者。所有患者均符合1990年美国风湿病学会对Takayasu动脉炎的标准。使用改良的美国国立卫生研究院评分评估疾病活动。结果：包括23例患者（女性14例，男性9例），平均年龄15.7±6.0岁。在23位患者中，有17位患者在IFX治疗之前使用了环磷酰胺，在CYC患者之前有23位患者使用了CYP之前的环磷酰胺。 CYC从疾病发作到开始治疗的平均时间为2.6±2.4年，而IFX为4.1±2.4年。 15例患者中有9例（60％）CYC失败，其中6例中的5例（83％）改用了IFX，随后临床稳定，由于缺乏适当的反应，最初接受IFX治疗的2例患者转用CYC：1病人后来恶化，另一名失去随访。 CYC组发生了五例需要住院的机会性感染，而IFX组中未发现任何机会性感染。与CYC患者相比，IFX组的患者更有可能减少或停止使用皮质类固醇。结论：环磷酰胺常被用作初始治疗，但有许多不良反应。在这个回顾性病例系列中，IFX等效于CYC，不良反应更少，这使得IFX成为pTA的替代治疗选择。

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