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Disease activity in primary progressive multiple sclerosis: a systematic review and meta-analysis
Katelijn M Blok1,2, Joost van Rosmalen3,4, Nura Tebayna1
1Department of Neurology, Albert Schweitzer Hospital, Dordrecht, Netherlands.
Background:
Disease activity in multiple sclerosis (MS) is defined as presence of relapses, gadolinium enhancing lesions and/or new or enlarging lesions on MRI. It is associated with efficacy of immunomodulating therapies (IMTs) in primary progressive MS (PPMS). However, a thorough review on disease activity in PPMS is lacking. In relapsing remitting MS, the prevalence of activity decreases in more contemporary cohorts. For PPMS, this is unknown.
Aim:
To review disease activity in PPMS cohorts and identify its predictors.
Methods:
A systematic search in EMBASE, MEDLINE, Web of science Core Collection, COCHRANE CENTRAL register of trials, and GOOGLE SCHOLAR was performed. Keywords included PPMS, inflammation, and synonyms. We included original studies with predefined available data, extracted cohort characteristics and disease activity outcomes and performed meta-regression analyses.
Results:
We included 34 articles describing 7,109 people with PPMS (pwPPMS). The weighted estimated proportion of pwPPMS with overall disease activity was 26.8% (95% CI 20.6-34.0%). A lower age at inclusion predicted higher disease activity (OR 0.91, p = 0.031). Radiological activity (31.9%) was more frequent than relapses (9.2%), and was predicted by longer follow-up duration (OR 1.27, p = 0.033). Year of publication was not correlated with disease activity.
Conclusion:
Inflammatory disease activity is common in PPMS and has remained stable over the last decades. Age and follow-up duration predict disease activity, advocating prolonged monitoring of young pwPPMS to evaluate potential IMT benefits.
Insights
Inflammatory disease activity is common in primary progressive multiple sclerosis (PPMS), affecting over a quarter of patients. Younger age at inclusion and longer follow-up predict disease activity, suggesting prolonged monitoring for potential treatment benefits.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Disease activity in multiple sclerosis (MS) is defined by relapses and MRI lesions.
- Understanding disease activity in primary progressive MS (PPMS) is crucial for treatment efficacy.
- Contemporary data on PPMS disease activity prevalence is lacking.
Approach:
- Systematic literature search across major databases (EMBASE, MEDLINE, Web of Science, Cochrane).
- Inclusion of original studies with predefined data on PPMS cohort characteristics and disease activity.
- Meta-regression analyses to identify predictors of disease activity.
Key Points:
- 34 articles and 7,109 individuals with PPMS were analyzed.
- Overall disease activity was observed in 26.8% of people with PPMS.
- Radiological activity (31.9%) was more common than relapses (9.2%).
Conclusions:
- Inflammatory disease activity is prevalent and stable in PPMS.
- Younger age at inclusion and longer follow-up duration predict disease activity.
- Prolonged monitoring of younger individuals with PPMS is recommended to assess treatment benefits.
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