From the Department of Internal Medicine (H.d.B., A.A.).
University of Caen-Basse Normandie, France (H.d.B., E.T., A.A.).
Stroke. 2018 Aug;49(8):1946-1952. doi: 10.1161/STROKEAHA.118.021878.
Background and Purpose- We aimed to analyze the long-term outcomes of patients with primary central nervous system vasculitis according to the different therapeutic strategies used to induce remission. Methods- We assessed the rate of prolonged remission (defined by the absence of relapse at ≥12 months after diagnosis) and the functional status at last follow-up in patients with primary central nervous system vasculitis included in the French cohort, who achieved a first remission according to the 3 main groups of treatments administered: glucocorticoids only (group 1); induction treatment with glucocorticoids and an immunosuppressant, but no maintenance (group 2); and combined treatment with glucocorticoids and an immunosuppressant for induction followed by maintenance therapy (group 3). Good functional status was defined as a modified Rankin Scale score ≤2 at the last follow-up. Results- Remission was achieved with the initial induction treatment in 106 (95%) of the 112. Prolonged remission without relapse was observed in 70 (66%) patients after 57 (12-198) months of follow-up. A good functional status at last follow-up (ie, modified Rankin Scale score ≤2) was observed in 63 (56%) patients. Overall mortality was 8%. The initial severity and the radiological presentations were comparable in the 3 treatment groups. More prolonged remissions ( P=0.003) and a better functional status at the last follow-up ( P=0.0004) were observed in group 3. In multivariate analysis, the use of maintenance therapy was associated with prolonged remission (odds ratio, 4.32 [1.67-12.19]; P=0.002) and better functional status (odds ratio, 8.09 [3.24-22.38]; P<0.0001). Conclusions- This study suggests that maintenance therapy with an immunosuppressant combined with glucocorticoids lead to the best long-term clinical and functional outcomes in patients with primary central nervous system vasculitis after having achieved remission with either glucocorticoids alone or in combination with another immunosuppressant.
背景与目的-我们旨在分析根据用于诱导缓解的不同治疗策略，原发性中枢神经系统血管炎患者的长期结局。方法-我们评估了原发性中枢神经系统血管炎患者的长期缓解率（定义为在诊断后≥12 个月无复发）和最后一次随访时的功能状态，这些患者在法国队列中根据以下 3 种主要治疗组之一达到了首次缓解：仅使用糖皮质激素（第 1 组）；使用糖皮质激素和免疫抑制剂诱导治疗，但无维持治疗（第 2 组）；以及使用糖皮质激素和免疫抑制剂联合诱导治疗，随后进行维持治疗（第 3 组）。功能状态良好定义为最后一次随访时改良 Rankin 量表评分≤2。结果-在 112 例患者中，有 106 例（95%）患者在初始诱导治疗中缓解。在 57（12-198）个月的随访后，70 例（66%）患者无复发的长期缓解。在最后一次随访时，63 例（56%）患者的功能状态良好（即改良 Rankin 量表评分≤2）。总死亡率为 8%。3 种治疗组的初始严重程度和影像学表现相似。第 3 组观察到更持久的缓解（P=0.003）和更好的最后随访时功能状态（P=0.0004）。多变量分析显示，维持治疗与延长缓解（优势比，4.32[1.67-12.19]；P=0.002）和更好的功能状态（优势比，8.09[3.24-22.38]；P<0.0001）相关。结论-这项研究表明，在原发性中枢神经系统血管炎患者使用糖皮质激素或联合另一种免疫抑制剂达到缓解后，使用免疫抑制剂联合糖皮质激素进行维持治疗可带来最佳的长期临床和功能结局。
