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Review
. 2014 Dec;16(12):477.
doi: 10.1007/s11926-014-0477-9.

Ongoing developments in sporadic inclusion body myositis

Pedro M Machado  1 , Mhoriam Ahmed, Stefen Brady, Qiang Gang, Estelle Healy, Jasper M Morrow, Amanda C Wallace, Liz Dewar, Gita Ramdharry, Matthew Parton, Janice L Holton, Henry Houlden, Linda Greensmith, Michael G Hanna
Affiliations
Review

Ongoing developments in sporadic inclusion body myositis

Pedro M Machado et al. Curr Rheumatol Rep. 2014 Dec.

Abstract

Sporadic inclusion body myositis (IBM) is an acquired muscle disorder associated with ageing, for which there is no effective treatment. Ongoing developments include: genetic studies that may provide insights regarding the pathogenesis of IBM, improved histopathological markers, the description of a new IBM autoantibody, scrutiny of the diagnostic utility of clinical features and biomarkers, the refinement of diagnostic criteria, the emerging use of MRI as a diagnostic and monitoring tool, and new pathogenic insights that have led to novel therapeutic approaches being trialled for IBM, including treatments with the objective of restoring protein homeostasis and myostatin blockers. The effect of exercise in IBM continues to be investigated. However, despite these ongoing developments, the aetiopathogenesis of IBM remains uncertain. A translational and multidisciplinary collaborative approach is critical to improve the diagnosis, treatment, and care of patients with IBM.

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Conflict of interest statement

Pedro Machado declares funding by a Post-Doctoral Research Fellowship award from the National Institute for Health Research (NIHR) (UK).

Mhoriam Ahmed declares support from the Brain Research Trust (UK).

Qiang Gang declares support from a University College London Impact Studentship and the Chinese Scholarship Council.

Amanda C. Wallace declares the she is the trial co-ordinator for a research trial: “Aerobic training in Charcot-Marie-Tooth and Inclusion Body Myositis,” funded by an NIHR (UK) Research for Patient Benefit grant.

Janice L. Holton declares support from Myositis UK.

Linda Greensmith is the Graham Watts Senior Fellow and declares support from Brain Research Trust (UK).

Michael G. Hanna declares support from a Medical Research Council (MRC) Centre grant 2013-18 (MR/K000608/01). The International IBM Consortium Genetic Study is supported by an MRC grant (MR/J004758/1). Dr Hanna is the UK Chief investigator of the Bimagrumab phase IIb and III trial (NCT01925209).

Stefen Brady, Estelle Healy, Jasper M. Morrow, Liz Dewar, Gita Ramdharry Matthew Parton, and Henry Houlden declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Typical MRI appearances in a patient with IBM. (a) Axial T1-weighted images of the mid-thigh (top), distal thigh (middle), and mid-calf (bottom). The thigh shows intramuscular fat accumulation, evident as hyperintensity; most notably within the quadriceps (RF: rectus femoris; VL: vastus lateralis; VM: vastus medialis), especially in the distal thigh. Hamstring involvement is asymmetric, with semimembranosus (SM) relatively spared on the left. In the calf the medial gastrocnemius (MG) is completely replaced by fat, with the soleus (So) also severely affected. (b) Axial T1-weighted image at mid-thigh of the same patient six years later shows significant progression of intramuscular fat accumulation, with only the biceps femoris (BF) relatively unaffected. (c) Axial STIR images at distal thigh (top) and mid-calf (calf) in the same patient at baseline. Acute muscle inflammation is evident as hyperintensity, most markedly in the vastus medialis and soleus
See this image and copyright information in PMC

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