Department of Rheumatology, Institute of Neurosciences Kolkata (I-NK), 185, Acharya Jagadish Chandra Bose Rd, Elgin, Kolkata, West Bengal, 700017, India.
Department of Rheumatology, All India Institute of Medical Sciences, Sri Aurobindo Marg, Ansari Nagar, Ansari Nagar East, Delhi, New Delhi, 110029, India.
Clin Rheumatol. 2023 Aug;42(8):2155-2162. doi: 10.1007/s10067-023-06602-y. Epub 2023 Apr 18.
To observe the clinical and angiographic effectiveness of mycophenolate mofetil (MMF) as induction and maintenance immunosuppressive therapy in primary central nervous system vasculitis (PCNSV). In this open-label prospective study done at a tertiary care neurology centre, adult patients with PCNSV, diagnosed by Calabrese's criteria, were recruited from 2017 to 2021 and treated with glucocorticoids, MMF and standard of care. Patients were followed-up and clinical and angiographic changes were recorded. Total 26 patients were recruited with median age 39 years (34-49) with a slight female predilection (61.5%). Angiographic diagnoses were: small vessels disease 11.5%; large vessels disease 42.3% and both in 46.2%. Median duration of follow-up was 24.5 months (14.25-38). Proportion of patients with severe disability (modified Rankin Score (mRS) 4-6) at baseline was 73.08% (19/26) which reduced to 7.69% (2/26) (p < 0.001). At the last follow-up mRS = 0 was achieved in 38.5% (10/26) and mRS of ≤ 1 was achieved in 69.2% (18/26). Median time to achieve a mRS ≤ 1 was 12 months (95% CI: 6.8-17.2). Angiography was repeated in 16 patients after a median duration of 13 months (10.5-19.7), out of which 10 (62.5%) showed improvement and 5 (31.2%) showed non-progression of lesions. MMF may be an effective immunosuppressive therapy in adult PCNSV as both induction and maintenance. Serial DSA of brain may be useful to monitor the effect of treatment. Key Points • Mycophenolate mofetil is effective as induction and maintenance immunosuppressive therapy in PCNSV. • Repeat angiogram may be useful to monitor treatment response in PCNSV.
观察吗替麦考酚酯（MMF）作为原发性中枢神经系统血管炎（PCNSV）诱导和维持免疫抑制治疗的临床和血管造影效果。在这项在三级护理神经病学中心进行的开放标签前瞻性研究中，2017 年至 2021 年间，根据 Calabrese 的标准诊断为 PCNSV 的成年患者接受了糖皮质激素、MMF 和标准治疗。对患者进行了随访，并记录了临床和血管造影变化。共招募了 26 名中位年龄 39 岁（34-49 岁）的患者，女性略占优势（61.5%）。血管造影诊断为：小血管疾病 11.5%；大血管疾病 42.3%，两者均为 46.2%。中位随访时间为 24.5 个月（14.25-38）。基线时严重残疾（改良 Rankin 评分（mRS）4-6）的患者比例为 73.08%（19/26），降至 7.69%（2/26）（p<0.001）。在最后一次随访时，26 名患者中的 38.5%（10/26）达到 mRS=0，69.2%（18/26）达到 mRS≤1。达到 mRS≤1 的中位时间为 12 个月（95%CI：6.8-17.2）。在中位时间 13 个月（10.5-19.7）后对 16 名患者重复进行了血管造影，其中 10 名（62.5%）显示改善，5 名（31.2%）显示病变无进展。MMF 可能是成人 PCNSV 的一种有效免疫抑制治疗方法，无论是诱导还是维持。脑连续 DSA 可能有助于监测治疗效果。要点 • 吗替麦考酚酯（MMF）作为原发性中枢神经系统血管炎（PCNSV）的诱导和维持免疫抑制剂治疗有效。 • 重复血管造影可能有助于监测 PCNSV 的治疗反应。
