Wolfson Neuroscience Laboratories, Faculty of Medicine, Imperial College London, Hammersmith Hospital Campus, 160 Du Cane Road, London, W12 0NN, UK.
Department of Medical Statistics, University Medical Center Göttingen, Humboltallee 32, 37073, Göttingen, Germany.
Drugs. 2017 May;77(8):885-910. doi: 10.1007/s40265-017-0726-0.
It is well recognised that the majority of the impact of multiple sclerosis (MS), both personal and societal, arises in the progressive phase where disability accumulates inexorably. As such, progressive MS (PMS) has been the target of pharmacological therapies for many years. However, there are no current licensed treatments for PMS. This stands in marked contrast to relapsing remitting MS (RRMS) where trials have resulted in numerous licensed therapies. PMS has proven to be a more difficult challenge compared to RRMS and this review focuses on secondary progressive MS (SPMS), where relapses occur before the onset of gradual, irreversible disability, and not primary progressive MS where disability accumulation occurs without prior relapses. Although there are similarities between the two forms, in both cases pinpointing when PMS starts is difficult in a condition in which disability can vary from day to day. There is also an overlap between the pathology of relapsing and progressive MS and this has contributed to the lack of well-defined outcomes, both surrogates and clinically relevant outcomes in PMS. In this review, we used the search term 'randomised controlled clinical drug trials in secondary progressive MS' in publications since 1988 together with recently completed trials where results were available. We found 34 trials involving 21 different molecules, of which 38% were successful in reaching their primary outcome. In general, the trials were well designed (e.g. double blind) with sample sizes ranging from 35 to 1949 subjects. The majority were parallel group, but there were also multi-arm and multidose trials as well as the more recent use of adaptive designs. The disability outcome most commonly used was the Expanded Disability Status Scale (EDSS) in all phases, but also magnetic resonance imaging (MRI)-measured brain atrophy has been utilised as a surrogate endpoint in phase II studies. The majority of the treatments tested in SPMS over the years were initially successful in RRMS. This has a number of implications in terms of targeting SPMS, but principally implies that the optimal strategy to target SPMS is to utilise the prodrome of relapses to initiate a therapy that will aim to both prevent progression and slow its accumulation. This approach is in agreement with the early targeting of MS but requires treatments that are both effective and safe if it is to be used before disability is a major problem. Recent successes will hopefully result in the first licensed therapy for PMS and enable us to test this approach.
众所周知,多发性硬化症(MS)的大多数影响,无论是个人还是社会层面的,都出现在疾病逐渐累积的进展期。因此,多年来,进展型多发性硬化症(PMS)一直是药物治疗的目标。然而,目前尚无针对 PMS 的获批治疗方法。这与复发缓解型多发性硬化症(RRMS)形成鲜明对比,后者的试验已产生了众多获批的治疗方法。与 RRMS 相比,PMS 更具挑战性,本综述重点关注继发性进展型多发性硬化症(SPMS),即疾病在逐渐恶化和不可逆转的残疾之前发生复发,而非原发性进展型多发性硬化症,后者的残疾累积是在没有先前复发的情况下发生的。尽管这两种形式有相似之处,但在残疾状况每天都可能变化的情况下,要确定 PMS 何时开始是很困难的。在 RRMS 和进展型 MS 的病理学之间也存在重叠,这导致 PMS 缺乏明确的替代终点和临床相关终点。在本综述中,我们使用了自 1988 年以来出版物中的搜索词“继发性进展型多发性硬化症的随机对照临床试验”,并结合了最近完成的有结果的试验。我们发现了 34 项涉及 21 种不同分子的试验,其中 38%的试验达到了主要终点。一般来说,这些试验设计良好(例如双盲),样本量范围为 35 至 1949 例。大多数是平行组试验,但也有双臂和多剂量试验,以及最近使用适应性设计。在所有阶段,最常用的残疾结局是扩展残疾状况量表(EDSS),但在 II 期研究中也使用磁共振成像(MRI)测量的脑萎缩作为替代终点。多年来,在 SPMS 中测试的大多数治疗方法最初在 RRMS 中是有效的。这在针对 SPMS 的靶向治疗方面具有多种影响,但主要意味着针对 SPMS 的最佳策略是利用复发的前驱期启动一种既能预防进展又能减缓其累积的治疗方法。这种方法与多发性硬化症的早期靶向治疗一致,但如果要在残疾成为主要问题之前使用,就需要有效的且安全的治疗方法。最近的成功有望为 PMS 的首个获批治疗方法奠定基础,并使我们能够测试这种方法。