National Referral Center for Rare Autoimmune and Systemic Diseases, Hopital Cochin, AP-HP, Université Paris Descartes, Paris, France.
Department of Internal Medecine, La Timone University Hospital, Marseille, France.
Autoimmun Rev. 2019 Jul;18(7):714-720. doi: 10.1016/j.autrev.2019.05.008. Epub 2019 May 4.
Age at onset of large-vessel vasculitis (LVV) is commonly used to distinguish giant cell arteritis (GCA) and Takayasu arteritis (TA). However, LVV between age 50 and 60 years may be difficult to classify.
We conducted a retrospective study including LVV aged between 50 and 60 years at onset (LVV, cases) and compared them to LVV aged over 60 years (LVV, controls). LVV was defined histologically and/or morphologically. Controls fulfilled ACR 1990 criteria for GCA or presented isolated aortitis.
We included 183 LVV and 183 gender-matched LVV. LVV had more frequent peripheral limb manifestations (23 vs. 5%), and less frequent cephalic (73 vs. 90%) and ocular signs (17 vs. 27%) than LVV. Compared to LVV, CT angiography and PET/CT scan were more frequently abnormal in LVV (74 vs. 38%, and 90 vs. 72%, respectively), with aorta being more frequently involved (78 vs. 47%). By multivariate analysis, absence of cephalic symptoms, presence of peripheral limb ischemia and aorta involvement, and increased CRP level were significantly associated with LVV presentation compared to LVV. At last follow-up, compared to LVV, LVV received significantly more lines of treatment (2 vs. 1), more frequent biologics (12 vs. 3%), had more surgery (10 vs. 0%), and had higher prednisone dose (8.8 vs. 6.5 mg/d) at last follow-up, CONCLUSION: LVV onset between 50 and 60 years identifies a subset of patients with more frequent aorta and peripheral vascular involvement and more refractory disease compared to patients with LVV onset after 60.
大血管血管炎(LVV)的发病年龄通常用于区分巨细胞动脉炎(GCA)和 Takayasu 动脉炎(TA)。然而,50 岁至 60 岁之间的 LVV 可能难以分类。
我们进行了一项回顾性研究,纳入了发病年龄在 50 岁至 60 岁之间的 LVV(LVV,病例组),并将其与发病年龄超过 60 岁的 LVV(LVV,对照组)进行比较。LVV 通过组织学和/或形态学定义。对照组符合 ACR 1990 年 GCA 标准或表现为孤立性主动脉炎。
我们纳入了 183 例 LVV 和 183 例性别匹配的 LVV。LVV 更常出现外周肢体表现(23% vs. 5%),而更少出现头部(73% vs. 90%)和眼部体征(17% vs. 27%)。与 LVV 相比,CT 血管造影和 PET/CT 扫描在 LVV 中更常异常(74% vs. 38%和 90% vs. 72%),且主动脉受累更常见(78% vs. 47%)。多变量分析显示,无头部症状、存在外周肢体缺血和主动脉受累、CRP 水平升高与 LVV 表现显著相关,而不是 LVV。末次随访时,与 LVV 相比,LVV 接受的治疗线数明显更多(2 条 vs. 1 条)、更频繁地使用生物制剂(12 例 vs. 3 例)、更多的手术(10 例 vs. 0 例)和更高的泼尼松剂量(8.8 毫克/天 vs. 6.5 毫克/天)。
50 岁至 60 岁之间 LVV 的发病提示与 60 岁以后发病的 LVV 患者相比,患者的主动脉和外周血管受累更频繁,疾病更难治疗。