Sahin Sezgin, Hopurcuoglu Duhan, Bektas Sule, Belhan Ezgi, Adrovic Amra, Barut Kenan, Canpolat Nur, Caliskan Salim, Sever Lale, Kasapcopur Ozgur
Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
Department of Pediatric Nephrology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
Int J Rheum Dis. 2019 Jan;22(1):132-139. doi: 10.1111/1756-185X.13425. Epub 2018 Nov 5.
To describe clinical manifestations, angiographic findings, treatment, activity and damage of our Takayasu arteritis patients.
The patients who met European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society classification criteria for childhood-onset Takayasu arteritis were reviewed in a retrospective longitudinal manner from 2002 to 2017. Extent of the disease was assessed by Disease Extent Index for Takayasu Arteritis (DEI.Tak), activity by Pediatric Vasculitis Activity Score and Indian Takayasu's Arteritis Activity Score (ITAS 2010) and damage by Pediatric Vasculitis Damage Index and Takayasu Arteritis Damage Score (TADS).
Sixteen subjects (75% female) with a median disease duration of 3.1 years were enrolled in this study. While the median age at disease onset was 12.1 years, there was only a 2.5 months diagnostic delay. Treatment regime included corticosteroids (100%), which were combined with azathioprine or methotrexate in 93.8% and 37.5% of the subjects, respectively. Severe and refractory cases were treated with cyclophosphamide (62.5%) and subsequently with tocilizumab (37.5%). Seven patients (43.8%) required either percutaneous endovascular intervention or bypass for severe disease refractory to medications. The correlation of the activity and damage scores with each other was fairly good. Damage was found to be associated only with high disease activity and extensive disease at disease onset, but not with other parameters.
Despite high usage rates of aggressive immunosuppressive therapy and biologic agents, almost half of the patients underwent interventional procedures. When medications failed, endovascular and surgical interventions were of great importance to avoid end-organ ischemia. The performance of the new activity (DEI.Tak and ITAS2010) and damage indices (TADS) seems satisfactory.
描述我们的大动脉炎患者的临床表现、血管造影结果、治疗、活动度及损害情况。
对2002年至2017年间符合欧洲抗风湿病联盟/儿科风湿病国际试验组织/欧洲儿科风湿病学会儿童期大动脉炎分类标准的患者进行回顾性纵向研究。采用大动脉炎疾病范围指数（DEI.Tak）评估疾病范围，采用儿科血管炎活动评分和印度大动脉炎活动评分（ITAS 2010）评估活动度，采用儿科血管炎损害指数和大动脉炎损害评分（TADS）评估损害情况。
本研究纳入了16名受试者（75%为女性），疾病中位病程为3.1年。疾病发病的中位年龄为12.1岁，诊断延迟仅2.5个月。治疗方案包括使用糖皮质激素（100%），分别有93.8%和37.5%的受试者联合使用硫唑嘌呤或甲氨蝶呤。重症和难治性病例接受环磷酰胺治疗（62.5%），随后使用托珠单抗治疗（37.5%）。7名患者（43.8%）因药物治疗难治的严重疾病需要进行经皮血管腔内介入治疗或搭桥手术。活动度评分与损害评分之间的相关性相当好。发现损害仅与疾病活动度高及疾病发病时病变广泛有关，而与其他参数无关。
尽管积极的免疫抑制治疗和生物制剂的使用率很高，但几乎一半的患者仍接受了介入治疗。当药物治疗无效时，血管腔内和外科干预对于避免终末器官缺血非常重要。新的活动度指标（DEI.Tak和ITAS2010）和损害指标（TADS）的表现似乎令人满意。
