A Spanish study assessed clinical and MRI features of patients who develop PIRA early on after the first attack of MS, or in the absence of recent inflammatory activity [1]. In a cohort of 754 patients with a clinically isolated syndrome (CIS) or early MS that were followed for >20 years, 209 (28%) developed PIRA after a median of 7.2 years. Presenting with PIRA after a CIS is not uncommon and implies an unfavourable, long-term prognosis, concluded Dr Carmen Tur (Vall d'Hebron University Hospital, Spain). Among all patients with PIRA, one third developed early PIRA, i.e. within 5 years after the CIS. Generally, patients with early PIRA were older at the time of CIS, with more spinal cord lesions and fewer relapses over time than late PIRA. Patients with late PIRA, on the other hand, were younger at CIS, with a higher inflammatory burden. “This suggests that late PIRA occurs as a predominantly inflammation-driven process.” Of all patients with PIRA, one third will develop “pure PIRA”, i.e. PIRA in the absence of recent inflammatory activity on MRI. Pure PIRA shares many characteristics with early PIRA. Dr Tur concluded that identifying those CIS patients who are at risk of developing any form of PIRA may have important therapeutical implications.
An Italian group investigated the rate and predictors of RAW and PIRA in paediatric (PO), adult (AO), and late-onset (LO) MS. In a relapsing MS cohort of 5,287 patients assessed within 1 year from onset, CDA events were more frequent in LOMS (61.3%) than in AOMS (45.9%) and POMS (41.0%; P<0.001) after a mean 11.4 years. PIRA accounted for about two-thirds of disability worsening, Dr Angelo Bellinvia (University of Florence, Italy) concluded. “Underlying MS progression is already detectable early in the disease course also in POMS patients and is more prevalent than RAW in AOMS and LOMS patients at the first demyelinating event. This data suggests that MS can be seen as a continuum from the very onset, and that age at onset seems to be one of the main determinants of CDA subtype, with RAW being more prevalent in POMS and PIRA in LOMS.” Early treatment with a disease-modifying therapy (DMT) was effective in preventing any CDA in all age groups and appeared to slow down the transition to the progressive phase. This underscores the importance of prompt initiation of DMTs in MS patients.
A Swiss group found that PIRA is associated with increased brain atrophy rates [3]. In a retrospective, longitudinal study including 516 relapsing-remitting MS patients, 334 were stable, 122 had relapsed, and 46 experienced PIRA. Both focal inflammatory activity and PIRA were associated with increased brain atrophy rates, without significant differences between the 2. The association between PIRA and diffuse neurodegeneration underscores the necessity to better stratify MS patients in clinical practice, as well as to optimise treatment to prevent accumulation of irreversible neuro-axonal loss.
- Tur C. Progression independent of relapse activity is present from disease onset: a complete view of the phenomenon in the CIS/early MS cohort of Barcelona. OP155, ECTRIMS 2021 Virtual Congress, 13–15 October.
- Bellinvia A. Progression independent of relapse activity in paediatric, adult and late-onset multiple sclerosis patients. OP154, ECTRIMS 2021 Virtual Congress, 13–15 October.
- Cagol A. Progression independent of relapse activity is associated with increased brain atrophy rates in patients with relapsing-remitting multiple sclerosis. OP156, ECTRIMS 2021 Virtual Congress, 13–15 October.
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Table of Contents: ECTRIMS 2021
Featured articles
Preliminary data shows positive results of ATA188 for progressive MS
COVID-19
MS patients at risk of hampered immune response after vaccination
Immunotherapy in MS does not influence COVID-19 severity and mortality
Anti-CD20 antibodies associated with worse COVID-19 outcomes
ECTRIMS-EAN consensus on vaccination in MS patients
Experimental Treatments
The role of astrocyte phenotypes in acute MS lesions
Promising results of intrathecal MSC-NTF cells in progressive MS
Preliminary data shows positive results of ATA188 for progressive MS
Evobrutinib reduces relapses and MRI lesion activity
Primary endpoint of opicinumab for relapsing MS not met in AFFINITY trial
Elezanumab did not outperform placebo in progressive and relapsing MS
Ibudilast reduced retinal atrophy in primary progressive MS
Treatment Trials and Strategies
ECTRIMS/EAN Clinical Guidelines on MS treatment: an update
Rituximab most effective initial MS therapy in Swedish real-world study
Ublituximab meets primary endpoint for relapsing MS
Dynamic scoring system aids decision to switch MS therapies early
Long-term suppression of MRI disease activity with ocrelizumab
Stopping DMT: when or if at all?
Biomarkers
Early predictors of disability progression in paediatric-onset MS
High-sensitive biomarker detection in MS via novel ELISA assay
Cortical lesions predict cognitive impairment 20 years after MS diagnosis
Applicability of sNfL measurement in clinical practice
MRI more sensitive for disease activity than relapses in SPMS
Imaging
Changes in GABA-receptor binding among cognitively impaired MS patients
T2 lesions independently predict early conversion to SPMS
Natural killer-like CD8+ T cells as a reservoir of clonal cells related to MS activity
Neuromyelitis Optica Spectrum Disorder (NMOSD)
Eculizumab, satralizumab, or inebilizumab for NMOSD?
Long-term efficacy of satralizumab for NMOSD
Long-term efficacy data: inebilizumab for NMOSD
Progressive MS
Charcot Award 2021: Progressive MS, a personal perspective
Top score poster: Meta-analysis on the effect of DMTs
Cortical lesions predict disease progression and disability accumulation
Ocrelizumab shows long-term benefits in primary progressive MS
Other
WNT9B-gene variant associated with doubled relapse risk in MS
Melatonin associated with improved sleep quality in MS patients
“Expanded Disability Status Scale 0 is not normal”
Personality trait alterations in MS patients
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