Inclusion body myositis (IBM) is a progressive inflammatory skeletal muscle disease of unknown cause and without effective treatment. This article discusses existing literature, emphasizing disease mechanisms and models. In particular, it addresses limitations in the beta-amyloid-mediated theory of IBM myofiber injury, flawed rationales of animal models of this disease, and recent reports regar...

Sporadic inclusion body myositis is a severely disabling myopathy. The design of effective treatment strategies is hampered by insufficient understanding of the complex disease pathology. Particularly, the nature of interrelationships between inflammatory and degenerative pathomechanisms in sporadic inclusion body myositis has remained elusive. In Alzheimer's dementia, accumulation of β-amyloid...

OBJECTIVE To analyze antisynthetase syndrome-associated myositis by modern myopathologic methods and to define its place in the spectrum of idiopathic inflammatory myopathies (IIMs). METHODS Skeletal muscle biopsies from antisynthetase syndrome-associated myositis and other IIMs from different institutions worldwide were analyzed by histopathology, quantitative PCR, and electron microscopy. ...

Objective: This report aimed to present a case of inclusion body myositis presenting with dysphagia and to review the literature. Methods: Case report and literature review. Results: Inclusion body myositis is a chronic progressive acquired myopathy, uniquely distinguished by its selective muscle involvement, normal or moderately elevated muscle enzyme concentrations, and a progressive corticos...

Sir, The recent Brain publication (Dalakas et al., 2009) describing a clinical study of alemtuzumab in patients with inclusion body myositis nicely demonstrates the feasibility of enrolling and following a cohort of these patients over a long period of time as participants in an intervention study. The authors state that ‘in [inclusion body myositis] one series of alemtuzumab infusions can slow...

The inflammatory myopathies, including dermatomyositis, inclusion body myositis, and polymyositis, are poorly understood autoimmune diseases affecting skeletal muscle. Dermatomyositis is a disease mainly of skin and muscle, but may affect lung and other tissues. Proximal or generalized weakness or skin rash are the typical presenting features. Inclusion body myositis has a specific clinical pat...

PURPOSE OF REVIEW This article reviews the results of recent therapeutic trials in dermatomyositis, polymyositis, and inclusion body myositis and suggests an approach to treating patients with inflammatory myopathy. RECENT FINDINGS We reviewed 10 double-blind, placebo-controlled therapeutic trials in patients with inflammatory myopathy. Only one, using intravenous immunoglobulin in refractory...