Standardized Definition of Progression Independent of Relapse Activity (PIRA) in Relapsing-Remitting Multiple Sclerosis

作者：Jannis Müller, Sifat Sharmin, Johannes Lorscheider, Serkan Özakbaş, Rana Karabudak, Dana Horáková, Bianca Weinstock‐Guttman, Vahid Shaygannejad, Masoud Etemadifar, Raed Alroughani, Francesco Patti, Sara Eichau, Alexandre Prat, Alessandra Lugaresi, Valentina Tomassini, Allan G. Kermode, Maria Pia Amato, Recai Türkoğlu, Ayşe Altıntaş, Katherine A. Buzzard, Aysun Soysal, Anneke van der Walt, Helmut Butzkueven, Yolanda Blanco, Oliver H. H. Gerlach, Samia Joseph Khoury, Michael J. Barnett, Nevin A. John, Jeannette Lechner‐Scott, Matteo Foschi, Andrea Surcinelli, Vincent Van Pesch, Julie Prévost, María José, Davide Maimone, Marie Beatrice D'hooghe, Stella Hughes, Suzanne J Hodgkinson, Christopher McGuigan, Elisabetta Cartechini, Bruce Taylor, Daniele L.A. Spitaleri, Mark Slee, Pamela McCombe, Bassem Yamout, Pascal Benkert, Jens Kühle, Ludwig Kappos, Izanne Roos, Tomáš Kalinčík, MSBase Study Group, Eva Havrdová, Marc Girard, Pierre Duquette, Marzena J. Fabis‐Pedrini, William M. Carroll, Olga G. Skibina, Riadh Gouider, Saloua Mrabet, Cristina M. Ramo-Tello, Claudio Marcello Solaro, Mario Habek, Bart V Wijmeersch, Radek Ampapa, Richard A L Macdonell, Celia Oreja‐Guevara, Koen de Gans, Guy Laureys, Jiwon Oh, Justin Garber, Orla M. Gray, Eduardo Agüera, José Luis Sánchez-Menoyo, Tamara Castillo‐Triviño, Nikolaos Grigoriadis, Thor Petersen, Todd A. Hardy, Steve Vucic, Stephen Reddel, Sudarshini Ramanathan, Abdullah Rashid Al-Asmi, Mihaela Adriana Simu, Seyed Mohammad Baghbanian, Dieter Poehlau, Talal Al‐Harbi, Juan Ignacio Rojas, Norma Deri, Patrice H. Lalive, Melissa Cambron, Tünde Csépány, Neil Shuey, Barbara M. P. Willekens, Cameron P. Shaw, D. Decoo, Jennifer C. Massey, Özgür Yaldizli, Tobias J Derfuss, Cristina Granziera · 发表于：JAMA Neurology · 年份：2025 · DOI：10.1001/jamaneurol.2025.0495 · 被引用次数：50 · 研究领域：Multiple Sclerosis Research Studies、Amyotrophic Lateral Sclerosis Research、Balance, Gait, and Falls Prevention

Importance: Progression independent of relapse activity (PIRA) is a significant contributor to long-term disability accumulation in relapsing-remitting multiple sclerosis (MS). Prior studies have used varying PIRA definitions, hampering the comparability of study results. Objective: To compare various definitions of PIRA. Design, Setting, and Participants: This cohort study involved a retrospective analysis of prospectively collected data from the MSBase registry from July 2004 to July 2023. The participants were patients with MS from 186 centers across 43 countries who had clinically definite relapsing-remitting MS, a complete minimal dataset, and 3 or more documented Expanded Disability Status Scale (EDSS) assessments. Exposure: Three-hundred sixty definitions of PIRA as combinations of the following criteria: baseline disability (fixed baseline with re-baselining after PIRA, or plus re-baselining after relapses, or plus re-baselining after improvements), minimum confirmation period (6, 12, or 24 months), confirmation magnitude (EDSS score at/above worsening score or at/above threshold compared with baseline), freedom from relapse at EDSS score worsening (90 days prior, 90 days prior and 30 days after, 180 days prior and after, since previous EDSS assessment, or since baseline), and freedom from relapse at confirmation (30 days prior, 90 days prior, 30 days before and after, or between worsening and confirmation). Main Outcome and Measure: For each definition, we quantified...