25 Feb 2024
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272nd ENMC international workshop: 10 Years of progress – revision of the ENMC 2013 diagnostic criteria for inclusion body myositis and clinical trial readiness. 16–18 June 2023, Hoofddorp, The Netherlands
Authors:
James B. Lilleker a,b,1, Elie Naddaf c,1, Christiaan G.J. Saris d,1, Jens Schmidt e,f,g, Marianne de
Visser h, Conrad C. Weihl i,∗, the 272nd ENMC workshop participants2
a Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK
b Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK
c Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
d Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands
e Department of Neurology and Pain Treatment, Neuromuscular Center and Center for Translational Medicine, Immanuel Klinik Rüdersdorf, University
Hospital of the Brandenburg Medical School MHB, Rüdersdorf bei Berlin, Germany
f Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany
g Department of Neurology, University Medical Center Göttingen, Göttingen, Germany
h Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands
i Neuromuscular Division, Washington University School of Medicine, Saint Louis, MO, USA
a
Since the publication of the 2013 European Neuromuscular Center (ENMC) diagnostic criteria for Inclusion Body Myositis (IBM), several advances have been made regarding IBM epidemiology, pathogenesis, diagnostic tools, and clinical trial readiness. Novel diagnostic tools include muscle imaging techniques such as MRI and ultrasound, and serological testing for cytosolic 5′ -nucleotidase-1A antibodies. The 272nd ENMC workshop aimed to develop new diagnostic criteria, discuss clinical outcome measures and clinical trial readiness. The workshop started with patient representatives highlighting several understudied symptoms and the urge for a timely diagnosis. This was followed by presentations from IBM experts
highlighting the new developments in the field. This report is composed of two parts, the first part providing new diagnostic criteria on which consensus was achieved. The second part focuses on the use of outcome measures in clinical practice and clinical trials, highlighting current limitations and outlining
the goals for future studies.