Abstract：
OBJECTIVE: We aimed to compare clinical spectrum and outcome between adults and children with Takayasu\'s arteritis (TAK) in a European population.
METHODS: We made a nationwide retrospective observational study between 1988 and 2019. All adult patients met the ACR diagnostic criteria for TAK and all children met the EULAR/PRINTO/PRES criteria for paediatric TAK.
RESULTS: We identified 46 children and 389 adults with TAK. The male to female ratio was 34/46 (0.74) in the paediatric group compared to 241/274 (0.88) in the adult group (P<0.05). Children presented with significantly more systemic symptoms; i.e., fever (P<0.05), fatigue (P<0.001), weight loss (P<0.001), abdominal pain (P<0.05), and myalgia (P<0.05) while adults had more upper limb claudication (P<0.01). Topography of the lesions differed significantly between the two groups: adults had more damage at the cerebral vasculature (P<0.01), upper and lower limbs (P<0.001) while children had more kidney lesions (P<0.05). Children TAK had more frequent (P<0.01) and higher (P<0.001) biological inflammation than adults. Children received higher dose-weight of corticosteroids (P=0.001) and less biotherapy (P<0.010) at diagnosis. Relapses (P<0.05) and death (8.6% vs 4.9%) were more frequent in children TAK than in adults.
CONCLUSIONS: Paediatric TAK seems more severe than adult TAK. Therefore, paediatrics patients may require closer monitoring and systemic use of biological treatment.
METHODS: We made a nationwide retrospective observational study between 1988 and 2019. All adult patients met the ACR diagnostic criteria for TAK and all children met the EULAR/PRINTO/PRES criteria for paediatric TAK.
RESULTS: We identified 46 children and 389 adults with TAK. The male to female ratio was 34/46 (0.74) in the paediatric group compared to 241/274 (0.88) in the adult group (P<0.05). Children presented with significantly more systemic symptoms; i.e., fever (P<0.05), fatigue (P<0.001), weight loss (P<0.001), abdominal pain (P<0.05), and myalgia (P<0.05) while adults had more upper limb claudication (P<0.01). Topography of the lesions differed significantly between the two groups: adults had more damage at the cerebral vasculature (P<0.01), upper and lower limbs (P<0.001) while children had more kidney lesions (P<0.05). Children TAK had more frequent (P<0.01) and higher (P<0.001) biological inflammation than adults. Children received higher dose-weight of corticosteroids (P=0.001) and less biotherapy (P<0.010) at diagnosis. Relapses (P<0.05) and death (8.6% vs 4.9%) were more frequent in children TAK than in adults.
CONCLUSIONS: Paediatric TAK seems more severe than adult TAK. Therefore, paediatrics patients may require closer monitoring and systemic use of biological treatment.
摘要：
目的：我们旨在比较欧洲人群中成人和儿童大动脉炎（TAK）的临床谱和结果。
方法：我们在1988年至2019年之间进行了一项全国性的回顾性观察研究。所有成人患者均符合TAK的ACR诊断标准，所有儿童均符合儿科TAK的EULAR/PRINTO/PRES标准。
结果：我们确定了46名儿童和389名成人患有TAK。儿科组的男女比例为34/46（0.74），而成年组为241/274（0.88）（p<0.05）。儿童表现出明显更多的全身症状；即发烧（p<0.05），疲劳(p<0.001)，体重减轻（p<0.001），腹痛（p<0.05），和肌痛（p<0.05），而成年人上肢跛行更多（p<0.01）。两组之间的病变形貌差异显着：成人在脑血管系统有更多的损伤（p<0.01），上肢和下肢（p<0.001），而儿童肾脏病变较多（p<0.05）。儿童TAK比成人具有更频繁(p<0.01)和更高(p<0.001)的生物学炎症。儿童在诊断时接受了较高剂量重量的皮质类固醇（p=0.001）和较少的生物治疗（p<0.010）。TAK儿童的复发（p<0.05）和死亡（8.6％vs4.9％）比成人更频繁。
结论：儿科TAK似乎比成人TAK更为严重。因此,儿科患者可能需要更密切的监测和系统使用生物治疗。
方法：我们在1988年至2019年之间进行了一项全国性的回顾性观察研究。所有成人患者均符合TAK的ACR诊断标准，所有儿童均符合儿科TAK的EULAR/PRINTO/PRES标准。
结果：我们确定了46名儿童和389名成人患有TAK。儿科组的男女比例为34/46（0.74），而成年组为241/274（0.88）（p<0.05）。儿童表现出明显更多的全身症状；即发烧（p<0.05），疲劳(p<0.001)，体重减轻（p<0.001），腹痛（p<0.05），和肌痛（p<0.05），而成年人上肢跛行更多（p<0.01）。两组之间的病变形貌差异显着：成人在脑血管系统有更多的损伤（p<0.01），上肢和下肢（p<0.001），而儿童肾脏病变较多（p<0.05）。儿童TAK比成人具有更频繁(p<0.01)和更高(p<0.001)的生物学炎症。儿童在诊断时接受了较高剂量重量的皮质类固醇（p=0.001）和较少的生物治疗（p<0.010）。TAK儿童的复发（p<0.05）和死亡（8.6％vs4.9％）比成人更频繁。
结论：儿科TAK似乎比成人TAK更为严重。因此,儿科患者可能需要更密切的监测和系统使用生物治疗。
