Division of Rheumatology and Clinical Hospital, Faculty of Medicine, University of São Paulo, Brazil.
Scand J Rheumatol. 2010 Nov;39(6):506-10. doi: 10.3109/03009741003742730. Epub 2010 Jun 21.
To compare prognosis parameters and arterial site involvement in Takayasu arteritis (TA) patients with disease onset at age ≤ 18 and ≥ 21 years.
Sixty-two TA patients [American College of Rheumatology (ACR) and European League Against Rheumatism/Paediatric Rheumatology European Society (EULAR/PreS) criteria] were enrolled consecutively and divided into two groups according to disease onset, and matched for disease duration: juvenile TA patients aged ≤ 18 years (n = 17) and adult TA patients aged ≥ 21 years (n = 45). The protocol evaluated the following prognostic factors: aortic insufficiency, ischaemic retinopathy, severe systemic hypertension, and arterial aneurysms. In addition, death and remission [defined as stable disease > 6 months (no complaints without immunosuppressive and prednisone use) and normal erythrocyte sedimentation rate (ESR)] were also analysed. Stenosis and aneurisms were investigated by magnetic angioresonance or arteriography and angiographic classification was defined according to Hata criteria.
Mean disease duration was similar in the juvenile and adult TA groups (13.50 ± 10.73 vs. 13.80 ± 7.17 years, p = 0.092) and a trend to a lower predominance of female gender in the juvenile TA group was observed (64.71% vs. 88.89%, p = 0.056). The prognosis was distinct in the two groups, with juvenile patients having a lower frequency of disease remission (23.53% vs. 55.56%, p = 0.04) and a significantly higher frequency of aneurism (41.0% vs. 11.1%, p = 0.013). Almost half of the juvenile TA patients had left renal stenosis, a frequency significantly higher than in the adult TA group (41.18% vs. 11.10%, p = 0.013), whereas the stenosis frequency was comparable in all other vascular sites evaluated. No differences were observed between the two groups regarding the frequency of aortic insufficiency, ischaemic retinopathy, severe systemic arterial hypertension, vascular procedures, and mortality. Angiographic classification revealed a similar distribution of arterial involvement in both groups (p > 0.05).
Juvenile TA patients have distinct characteristics, with a peculiar renal vascular involvement, the presence of aneurism, and a more refractory disease compared with adult TA patients.
比较发病年龄≤18 岁和≥21 岁的 Takayasu 动脉炎（TA）患者的预后参数和动脉受累部位。
连续纳入 62 例 TA 患者（美国风湿病学会[ACR]和欧洲抗风湿病联盟/儿科风湿病欧洲学会[EULAR/PreS]标准），并根据发病年龄分为两组，并进行疾病持续时间匹配：≤18 岁的青少年 TA 患者（n=17）和≥21 岁的成年 TA 患者（n=45）。该方案评估了以下预后因素：主动脉瓣关闭不全、缺血性视网膜病变、严重系统性高血压和动脉动脉瘤。此外，还分析了死亡和缓解[定义为稳定疾病>6 个月（无投诉，无免疫抑制和泼尼松治疗）和正常红细胞沉降率（ESR）]。狭窄和动脉瘤通过磁共振血管成像或血管造影检查，根据 Hata 标准进行血管造影分类。
青少年 TA 组和成年 TA 组的平均疾病持续时间相似（13.50±10.73 与 13.80±7.17 年，p=0.092），并且青少年 TA 组女性性别比例较低（64.71%与 88.89%，p=0.056）。两组的预后明显不同，青少年患者疾病缓解率较低（23.53%与 55.56%，p=0.04），动脉瘤发生率显著较高（41.0%与 11.1%，p=0.013）。几乎一半的青少年 TA 患者存在左肾狭窄，这一频率明显高于成年 TA 组（41.18%与 11.10%，p=0.013），而所有其他评估的血管部位的狭窄频率相当。两组之间主动脉瓣关闭不全、缺血性视网膜病变、严重系统性高血压、血管介入和死亡率的频率无差异。血管造影分类显示两组动脉受累分布相似（p>0.05）。
青少年 TA 患者具有独特的特征，与成年 TA 患者相比，具有独特的肾血管受累、动脉瘤的存在以及更难治性的疾病。
