Elsevier

Neuromuscular Disorders

Volume 20, Issue 2, February 2010, Pages 142-147
Neuromuscular Disorders

Workshop report
Inclusion body myositis: MRC Centre for Neuromuscular Diseases, IBM workshop, London, 13 June 2008

https://doi.org/10.1016/j.nmd.2009.11.003Get rights and content

Introduction

This first MRC workshop on inclusion body myositis assembled 29 clinicians and scientists. The workshop sought to establish a platform upon which an organised international network of neuromuscular specialists could consider a range of important unresolved issues in relation to IBM. The aims of the workshop were as follows: to review current practice regarding clinical and histopathological diagnostic criteria, to assess recent advances in relation to aetiology and basic science with potential for translation into clinical trials, to review current evidence for IBM treatments and lessons applicable to future trial design, to review genetic advances in IBM and to consider an IBM disease-specific prospective database in an electronic network.

Section snippets

Consideration of clinical diagnostic criteria

After a brief discussion of the typical clinical features of IBM [1], [2], [3], [4], David Hilton-Jones updated the workshop on the current diagnostic criteria for IBM [5], [6], [7], [8]. The widely employed Griggs criteria for ‘definite’ IBM requires fulfilment of several histopathological criteria encompassing degenerative and inflammatory disease processes [5]. According to the Griggs criteria the diagnosis can be considered definite on the basis of fulfilling certain histopathological

Aetiology of IBM

Adrian Miller summarized the evidence underlying the pathogenesis of IBM.

In sIBM, inflammation is manifested by clonally expanded CD8+ T-cells invading fibres expressing MHC Class I. Additional associations with autoimmune-prone haplotypes, paraproteinaemia, and the upregulation of cytokines imply an immunopathogenic component. The coexistence of an IBM-like myopathy with HIV and HTLV-1 infection [10] might imply a viral trigger to autoimmunity in sIBM. Analogous to Alzheimer’s disease, sIBM

Review of evidence for IBM treatments and lessons applicable to future trial design

Michael Rose summarized the existing evidence for IBM treatments. The majority of drug treatments have targeted inflammatory and immune mechanisms. Other trials have included antioxidants and mitochondrial impairment (carnitine and ubiquinone). Nine randomized control trials in IBM have been identified for a Cochrane Systematic Review in press. In addition to being relatively few in number, most have recruited small numbers of patients. Other than those studies coordinated by the north American

Genome-wide studies in IBM

Nick Wood described the options for a genetic approach to complex traits such as sporadic IBM. Whilst associations have been demonstrated between IBM and HLA alleles [31], [32], [33], [34], [35], it remains undetermined whether the predilection of IBM for certain muscle groups or the apparent deficiencies of cytoprotective machinery reflects genetic susceptibility. Possible genetic studies include linkage based or candidate gene studies and more modern techniques such as a genome wide

IBM prospective cohort database

Matt Parton described the full details of the new ‘IBM-net’ database which will represent a structured collection of clinical records and a means to prospectively collect store and retrieve patient data according to agreed protocol. The protocol was presented outlining a range of fields that would be collected. The system is ready to be set up on-line and agreement has been reached with a UK IT provider for this purpose. Potential harmonization with other EU-networks including TREAT-NMD has

List of participants

Mhoriam Ahmed,London, UK
Charlotte Brierley,Cambridge, UK
Linda Greensmith,London, UK
Simon Hammans,Southampton, UK
Michael Hanna,London, UK
David Hilton-Jones,Oxford, UK
Janice Holton,London, UK
Henry Houlden,London, UK
Russell Lane,London, UK
Hanns Lochmuller,Newcastle, UK
Adnan Manzur,London, UK
Adrian Miller,London, UK
James Miller,Newcastle, UK
Francesco Muntoni,London, UK
Fiona Norwood,London, UK
Irene Oakley,Myositis Support Group, UK
Richard Orrell,London, UK
Matt Parton,London, UK
Aleks Radunovic,

Acknowledgement

This IBM workshop was supported by the MRC Centre for Neuromuscular Diseases (Centre Grant G0601943).

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      IBM diagnosis was always confirmed with a muscle biopsy. The histological biopsy for the diagnosis of IBM was routinely taken from the vastus lateralis muscle (n = 37), and the muscle biopsy findings were compatible with IBM according to the Griggs' or ENMC criteria [26–27]. The histological features for the diagnosis of IBM have been reported earlier [18].

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