| Resumen: |
This cohort study investigates the long-term outcomes of patients who develop progression independent of relapse activity after a first demyelinating event in multiple sclerosis. What are the long-term outcomes of patients developing progression independent of relapse activity (PIRA) after a first demyelinating event in multiple sclerosis? In this longitudinal cohort study including 1128 patients with a first demyelinating event in multiple sclerosis, presenting with PIRA was associated with significantly shorter times to developing severe disability compared with not presenting with PIRA. Patients presenting with PIRA within the first 5 years of multiple sclerosis had a significantly 26-fold greater risk of developing severe disability than patients whose first PIRA appeared late in the disease. Results suggest that presenting with PIRA after a first demyelinating event in multiple sclerosis is an ominous prognosis, especially if it occurs early in the disease course. Progression independent of relapse activity (PIRA) is the main event responsible for irreversible disability accumulation in relapsing multiple sclerosis (MS). To investigate clinical and neuroimaging predictors of PIRA at the time of the first demyelinating attack and factors associated with long-term clinical outcomes of people who present with PIRA. This cohort study, conducted from January 1, 1994, to July 31, 2021, included patients with a first demyelinating attack from multiple sclerosis; patients were recruited from 1 study center in Spain. Patients were excluded if they refused to participate, had alternative diagnoses, did not meet protocol requirements, had inconsistent demographic information, or had less than 3 clinical assessments. Exposures included (1) clinical and neuroimaging features at the first demyelinating attack and (2) presenting PIRA, ie, confirmed disability accumulation (CDA) in a free-relapse period at any time after symptom onset, within (vs after) the first 5 years of the disease (ie, early/late PIRA), and in the presence (vs absence) of new T2 lesions in the previous 2 years (ie, active/nonactive PIRA). Expanded Disability Status Scale (EDSS) yearly increase rates since the first attack and adjusted hazard ratios (HRs) for predictors of time to PIRA and time to EDSS 6. 0. Of the 1128 patients (mean [SD] age, 32. 1 [8. 3] years; 781 female individuals [69. 2%]) included in the study, 277 (25%) developed 1 or more PIRA events at a median (IQR) follow-up time of 7. 2 (4. 6-12. 4) years (for first PIRA). Of all patients with PIRA, 86 of 277 (31%) developed early PIRA, and 73 of 144 (51%) developed active PIRA. Patients with PIRA were slightly older, had more brain lesions, and were more likely to have oligoclonal bands than those without PIRA. Older age at the first attack was the only predictor of PIRA (HR, 1. 43; 95% CI, 1. 23-1. 65; P <. 001 for each older decade). Patients with PIRA had steeper EDSS yearly increase rates (0. 18; 95% CI, 0. 16-0. 20 vs 0. 04; 95% CI, 0. 02-0. 05; P < . 001) and an 8-fold greater risk of reaching EDSS 6. 0 (HR, 7. 93; 95% CI, 2. 25-27. 96; P = . 001) than those without PIRA. Early PIRA had steeper EDSS yearly increase rates than late PIRA (0. 31; 95% CI, 0. 26-0. 35 vs 0. 13; 95% CI, 0. 10-0. 16; P < . 001) and a 26-fold greater risk of reaching EDSS 6. 0 from the first attack (HR, 26. 21; 95% CI, 2. 26-303. 95; P = . 009). Results of this cohort study suggest that for patients with multiple sclerosis, presenting with PIRA after a first demyelinating event was not uncommon and suggests an unfavorable long-term prognosis, especially if it occurs early in the disease course. |
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identificación
(Hospital Universitari Vall d'Hebron)
