Objectives: To compare the presenting features and outcomes of patients with cranial-limited (C-) and large-vessel (LV-) giant cell arteritis (GCA).

Methods: Data from our GCA cohort were collected retrospectively. Patients who underwent total-body large-vessel imaging within 10 days after commencing steroid therapy were included. Patients with LV involvement were classified as LV-GCA. Presenting features, treatments, and outcomes of LV-GCA and C-GCA patients were compared.

Results: 161 patients were included (LV-GCA, n = 100). At baseline, LV-GCA patients were younger than those with C-GCA (73.2 ± 8.9 vs 76 ± 8.8 years, p = 0.018) and had a longer delay to diagnosis (3.5 ± 4.6 vs 2.3 ± 4.9 months, p = 0.001). C-GCA patients had a higher incidence of headache (p = 0.006) and ischaemic optic neuropathy (p < 0.001), whereas LV-GCA patients had more systemic symptoms (fever, p = 0.002; fatigue, p < 0.001; weight loss, p < 0.001; night sweats, p = 0.015) and dry cough (p = 0.031). Corrected cumulative prednisone dose, relapse-free survival, relapse-rate, and incidence of ascending aortic aneurysms were not significantly different between the two subgroups. A steroid-sparing agent was added in 73% of LV- and 55.7% of C-GCA patients (p = 0.027), but was introduced more frequently at baseline in LV-GCA patients (52% vs 23.5%, p = 0.006). LV-GCA patients initially treated with glucocorticoid monotherapy relapsed sooner (relapse-free survival, HR = 0.56, 95% CI 0.41–0.78, p < 0.001) and had a higher relapse rate (relapses per 10 person-years, 6.73 ± 11.50 vs 3.82 ± 10.83, p = 0.011).

Conclusion: LV-GCA patients were younger at diagnosis and suffered a longer diagnostic delay. The outcomes of the two subgroups were similar. An earlier introduction of steroid-sparing agents in LV-GCA patients might have played a positive role.

目的：比较颅受限 (C-) 和大血管 (LV-) 巨细胞动脉炎 (GCA) 患者的表现特征和结果。

方法：回顾性收集来自我们的 GCA 队列的数据。包括在开始类固醇治疗后 10 天内接受全身大血管成像的患者。LV 受累的患者被归类为 LV-GCA。比较了 LV-GCA 和 C-GCA 患者的表现特征、治疗和结果。

结果：包括 161 名患者（LV-GCA，n = 100）。在基线时，LV-GCA 患者比 C-GCA 患者年轻（73.2 ± 8.9 对 76 ± 8.8 岁，p = 0.018）并且诊断延迟更长（3.5 ± 4.6 vs 2.3 ± 4.9 个月，p = 0.001） . C-GCA 患者的头痛 (p = 0.006) 和缺血性视神经病变 (p < 0.001) 的发生率较高，而 LV-GCA 患者的全身症状更多（发热，p = 0.002；疲劳，p < 0.001；体重减轻， p < 0.001；盗汗，p = 0.015）和干咳（p = 0.031）。校正的累积泼尼松剂量、无复发生存期、复发率和升主动脉瘤发生率在两个亚组之间没有显着差异。73% 的 LV-和 55.7% 的 C-GCA 患者添加了类固醇保留剂（p = 0.027），但在 LV-GCA 患者的基线时更频繁地使用（52% vs 23. 5%，p = 0.006）。最初接受糖皮质激素单药治疗的 LV-GCA 患者复发更快（无复发生存期，HR = 0.56, 95% CI 0.41–0.78, p < 0.001）并且复发率更高（每 10 人年复发，6.73 ± 11.50 vs 3.82 ± 10.83，p = 0.011）。

结论： LV-GCA 患者在诊断时更年轻，诊断延迟时间更长。两个亚组的结果相似。早期在 LV-GCA 患者中引入类固醇节约剂可能起到了积极作用。