Chisari Clara Grazia, Amato Maria Pia, Di Sapio Alessia, Foschi Matteo, Iaffaldano Pietro, Inglese Matilde, Fermo Salvatore Lo, Lugaresi Alessandra, Lus Giacomo, Mascoli Nerina, Montepietra Sara, Pesci Ilaria, Quatrale Rocco, Salemi Giuseppe, Torri Clerici Valentina, Totaro Rocco, Valentino Paola, Filippi Massimo, Patti Francesco
Department of Medical and Surgical Sciences and Advanced Technologies "GF Ingrassia", Multiple Sclerosis Center, University of Catania, Catania, Italy.
Multiple Sclerosis Unit; Neurology Clinic, Policlinico "G. Rodolico- San Marco", Catania, Italy.
J Neurol. 2024 Oct;271(10):6801-6810. doi: 10.1007/s00415-024-12621-9. Epub 2024 Aug 27.
'Active' and 'non-active' secondary progressive MS (SPMS) have distinct pathophysiological mechanisms and clinical characteristics, but there is still no consensus regarding the frequency of these MS forms in the real-world setting. We aimed to evaluate the frequency of 'active' and 'non-active' SPMS in a large cohort of Italian MS patients and the differences in terms of clinical and MRI characteristics and disease progression. This multicenter study collected data about MS patients who have transitioned to the SP form in the period between 1st January 2014 and 31st December 2019 and followed by the MS centers contributing to the Italian MS Registry. Patients were divided into 'active SPMS' and 'non-active SPMS', based on both reported MRI data and relapse activity in the year before conversion to SPMS. Out of 68,621, 8,316 (12.1%) patients were diagnosed with SPMS. Out of them, 872 (10.5%) were classified into patients with either 'active' or 'non-active' SPMS. A total of 237 were classified into patients with 'active SPMS' (27.2%) and 635 as 'non-active SPMS' (72.8%). 'Non-active SPMS' patients were older, with a longer disease duration compared to those with 'active SPMS'. The percentages of patients showing progression independent of relapse activity (PIRA) at 24 months were similar between 'active' and 'non-active' SPMS patients (67 [27.4%] vs 188 [29.6%]; p = 0.60). In the 'active' group, 36 (15.2%) patients showed relapse-associated worsening (RAW). Comparison of the survival curves to EDSS 6 and 7 according to disease activity did not show significant differences (p = 0.68 and p = 0.71). 'Active' and 'non-active' SPMS patients had a similar risk of achieving disability milestones, suggesting that progression is primarily attributed to PIRA and only to a small extent to disease activity.
“活动性”和“非活动性”继发进展型多发性硬化(SPMS)具有不同的病理生理机制和临床特征,但在现实环境中这些MS形式的发生率仍未达成共识。我们旨在评估一大群意大利MS患者中“活动性”和“非活动性”SPMS的发生率,以及临床和MRI特征及疾病进展方面的差异。这项多中心研究收集了2014年1月1日至2019年12月31日期间转变为SP形式并由参与意大利MS注册中心的MS中心随访的MS患者的数据。根据报告的MRI数据和转变为SPMS前一年的复发活动情况,将患者分为“活动性SPMS”和“非活动性SPMS”。在68621名患者中,8316名(12.1%)被诊断为SPMS。其中,872名(10.5%)被分类为“活动性”或“非活动性”SPMS患者。共有237名被分类为“活动性SPMS”患者(27.2%),635名被分类为“非活动性SPMS”患者(72.8%)。“非活动性SPMS”患者年龄更大,与“活动性SPMS”患者相比疾病持续时间更长。“活动性”和“非活动性”SPMS患者在24个月时显示出与复发活动无关的疾病进展(PIRA)的患者百分比相似(67名[27.4%]对188名[29.6%];p = 0.60)。在“活动性”组中,36名(15.2%)患者显示出与复发相关的病情恶化(RAW)。根据疾病活动情况将生存曲线与EDSS 6和7进行比较未显示出显著差异(p = 0.68和p = 0.71)。“活动性”和“非活动性”SPMS患者达到残疾里程碑的风险相似,这表明疾病进展主要归因于PIRA,仅在很小程度上归因于疾病活动。