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Review

From Phenotypes to Spectrum: Rethinking RRMS, SPMS and PPMS in the Era of PIRA—A Framework Integrating PIRA, Smouldering-Associated Worsening, and Neurologic Reserve to Facilitate Earlier Recognition of Progression

1
Clinic for Movement Disorders, University Multiprofile Hospital for Treatment in Neurology and Psychiatry “St. Naum”, 1113 Sofia, Bulgaria
2
Department of Neurology, Medical University, 1431 Sofia, Bulgaria
3
Clinic of Neurology, Multiprofile Hospital for Active Treatment “Uni Hospital”, 4500 Panagyurishte, Bulgaria
*
Author to whom correspondence should be addressed.
Neurol. Int. 2026, 18(5), 86; https://doi.org/10.3390/neurolint18050086
Submission received: 3 April 2026 / Revised: 26 April 2026 / Accepted: 27 April 2026 / Published: 2 May 2026
(This article belongs to the Special Issue Underlying Signalings in the Neuro-Immune Communications)

Abstract

The conventional classification of multiple sclerosis (MS) into relapsing–remitting, secondary progressive, and primary progressive phenotypes has long guided diagnosis, prognosis, and therapeutic decision-making. However, accumulating evidence indicates that disability accumulation frequently occurs independently of clinical relapses, challenging relapse-centric and phenotype-based models of disease evolution. The concept of progression independent of relapse activity (PIRA) has emerged as a clinically relevant framework capturing this phenomenon across MS phenotypes. In this state-of-the-art narrative review, we propose a spectrum-based reinterpretation of MS, integrating PIRA with concepts of smouldering-associated worsening and neurologic reserve. We highlight the heterogeneity of relapse-independent worsening, distinguishing transient from persistent PIRA, and discuss how ageing-related decline in compensatory capacity contributes to the clinical unmasking of progression over time. Within this framework, secondary progressive MS is redefined as the clinically recognizable accumulation of persistent relapse-independent worsening, while primary progressive MS is conceptualized as early predominance of clinically manifest progression due to limited reserve rather than a distinct disease entity. Finally, we examine diagnostic and therapeutic implications of a spectrum-based model in the contemporary era, emphasizing the limitations of relapse-centric treatment strategies and unmet needs in addressing progression-related biology. By reframing MS as a dynamic continuum shaped by the interaction between ongoing pathology and evolving neurologic reserve, this review aims to support earlier recognition of clinically meaningful progression and to inform more biology-aware approaches to disease monitoring and therapy.
Keywords: multiple sclerosis; progression independent of relapse activity (PIRA); disability progression; neurologic reserve; biological ageing; smouldering disease activity; neuroaxonal injury; disease continuum; phenotype reclassification multiple sclerosis; progression independent of relapse activity (PIRA); disability progression; neurologic reserve; biological ageing; smouldering disease activity; neuroaxonal injury; disease continuum; phenotype reclassification
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MDPI and ACS Style

Vasilev, G.V.; Ivanova, S.; Milanov, I. From Phenotypes to Spectrum: Rethinking RRMS, SPMS and PPMS in the Era of PIRA—A Framework Integrating PIRA, Smouldering-Associated Worsening, and Neurologic Reserve to Facilitate Earlier Recognition of Progression. Neurol. Int. 2026, 18, 86. https://doi.org/10.3390/neurolint18050086

AMA Style

Vasilev GV, Ivanova S, Milanov I. From Phenotypes to Spectrum: Rethinking RRMS, SPMS and PPMS in the Era of PIRA—A Framework Integrating PIRA, Smouldering-Associated Worsening, and Neurologic Reserve to Facilitate Earlier Recognition of Progression. Neurology International. 2026; 18(5):86. https://doi.org/10.3390/neurolint18050086

Chicago/Turabian Style

Vasilev, Georgi V., Sonya Ivanova, and Ivan Milanov. 2026. "From Phenotypes to Spectrum: Rethinking RRMS, SPMS and PPMS in the Era of PIRA—A Framework Integrating PIRA, Smouldering-Associated Worsening, and Neurologic Reserve to Facilitate Earlier Recognition of Progression" Neurology International 18, no. 5: 86. https://doi.org/10.3390/neurolint18050086

APA Style

Vasilev, G. V., Ivanova, S., & Milanov, I. (2026). From Phenotypes to Spectrum: Rethinking RRMS, SPMS and PPMS in the Era of PIRA—A Framework Integrating PIRA, Smouldering-Associated Worsening, and Neurologic Reserve to Facilitate Earlier Recognition of Progression. Neurology International, 18(5), 86. https://doi.org/10.3390/neurolint18050086

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