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进行性多发性硬化症。

Progressive Multiple Sclerosis.

出版信息

Continuum (Minneap Minn). 2022 Aug 1;28(4):1083-1103. doi: 10.1212/CON.0000000000001157.

Abstract

PURPOSE OF REVIEW

This article provides an update on progressive forms of multiple sclerosis (MS) commonly referred to as primary progressive MS and secondary progressive MS. It discusses the importance of diagnosing and detecting progression early, the similarities between progressive forms, challenges in detecting progression, factors that could augment progression, and the importance of disease-modifying therapies in patients with evidence of active progressive MS. It also discusses the overall care of progressive MS.

RECENT FINDINGS

The pathogenesis of primary progressive MS and secondary progressive MS is overlapping, and in both presentations, patients with relapses or focal MRI activity are classified as having active, progressive MS. All currently approved disease-modifying therapies are indicated for active secondary progressive MS. The therapeutic opportunity of anti-inflammatory drugs for the treatment of progressive MS is enhanced in those who are younger and have a shorter disease duration. Vascular comorbidities may contribute to progression in MS.

SUMMARY

Several challenges remain in the diagnosis, follow-up, and treatment of progressive MS. Early identification of active progressive MS is needed to maximize treatment benefit. The advantages of optimal comorbidity management (eg, hypertension, hyperlipidemia) in delaying progression are uncertain. Clinical care guidelines for advanced, severe MS are lacking.

摘要

目的综述

本文介绍了多发性硬化症(MS)的进行性形式,通常称为原发性进行性 MS 和继发性进行性 MS。讨论了早期诊断和检测进展的重要性、进行性形式之间的相似性、检测进展的挑战、可能增强进展的因素,以及在有活动性进行性 MS 证据的患者中使用疾病修正疗法的重要性。还讨论了进行性 MS 的整体护理。

最新发现

原发性进行性 MS 和继发性进行性 MS 的发病机制是重叠的,在这两种表现中,有复发或局灶性 MRI 活动的患者被归类为具有活动性、进行性 MS。所有目前批准的疾病修正疗法都适用于活动性继发性进行性 MS。在年龄较小和疾病持续时间较短的患者中,抗炎药物治疗进行性 MS 的治疗机会更大。血管合并症可能导致 MS 进展。

总结

在进行性 MS 的诊断、随访和治疗方面仍存在一些挑战。需要早期识别活动性进行性 MS,以最大限度地提高治疗效果。最佳合并症管理(如高血压、高血脂)在延缓进展方面的优势尚不确定。晚期、严重 MS 的临床护理指南仍缺乏。

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