BACKGROUND No uniform criteria for a sensitive identification of the transition from relapsing-remitting multiple sclerosis (MS) to secondary-progressive multiple sclerosis (SPMS) are available. OBJECTIVE To compare risk factors of SPMS using two definitions: one based on the neurologist judgment (ND) and an objective data-driven algorithm (DDA). METHODS Relapsing-onset MS patients (n = 19,318) were extracted from the Italian MS Registry. Risk factors for SPMS and for reaching irreversible Expanded Disability Status Scale (EDSS) 6.0, after SP transition, were estimated using multivariable Cox regression models. RESULTS SPMS identified by the DDA (n = 2343, 12.1%) were older, more disabled and with a faster progression to severe disability (p < 0.0001), than those identified by the ND (n = 3868, 20.0%). In both groups, the most consistent risk factors (p < 0.05) for SPMS were a multifocal onset, an age at onset >40 years, higher baseline EDSS score and a higher number of relapses; the most consistent protective factor was the disease-modifying therapy (DMT) exposure. DMT exposure during SP did not impact the risk of reaching irreversible EDSS 6.0. CONCLUSION A DDA definition of SPMS identifies more aggressive progressive patients. DMT exposure reduces the risk of SPMS conversion, but it does not prevent the disability accumulation after the SP transition.

中文翻译：

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复发性多发性硬化症向继发性进展的转变：定义和危险因素

背景技术对于从复发缓解型多发性硬化症 (MS) 到继发性进行性多发性硬化症 (SPMS) 的转变，没有统一的标准可用于敏感识别。目的 使用两种定义比较 SPMS 的风险因素：一种基于神经学家判断 (ND) 和客观数据驱动算法 (DDA)。方法 从意大利 MS 登记处提取复发性 MS 患者（n = 19,318）。使用多变量 Cox 回归模型估计 SPMS 和达到不可逆扩展残疾状态量表 (EDSS) 6.0 的风险因素，在 SP 过渡后。结果 DDA 确定的 SPMS (n = 2343, 12.1%) 比 ND 确定的 SPMS (n = 3868, 20.0%) 年龄更大，残疾更多，并且进展为严重残疾的速度更快 (p < 0.0001)。在两组中，SPMS 最一致的风险因素 (p < 0.05) 是多灶性发病、发病年龄 > 40 岁、基线 EDSS 评分较高和复发次数较多；最一致的保护因素是疾病缓解疗法 (DMT) 暴露。SP 期间的 DMT 暴露不会影响达到不可逆 EDSS 6.0 的风险。结论 SPMS 的 DDA 定义可识别更具侵略性的进展患者。DMT 暴露降低了 SPMS 转换的风险，但并不能阻止 SP 转换后的残疾积累。SP 期间的 DMT 暴露不会影响达到不可逆 EDSS 6.0 的风险。结论 SPMS 的 DDA 定义可识别更具侵略性的进展患者。DMT 暴露降低了 SPMS 转换的风险，但并不能阻止 SP 转换后的残疾积累。SP 期间的 DMT 暴露不会影响达到不可逆 EDSS 6.0 的风险。结论 SPMS 的 DDA 定义可识别更具侵略性的进展患者。DMT 暴露降低了 SPMS 转换的风险，但并不能阻止 SP 转换后的残疾积累。