Ongoing Developments in Sporadic Inclusion Body Myositis
نویسندگان
چکیده
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.
منابع مشابه
Inclusion body myositis.
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare disorders that share many similarities. In addition to sporadic inclusion body myositis (IBM), these include dermatomyositis, polymyositis, and autoimmune necrotizing myopathy. IBM is the most common IIM after age 50 years. Muscle histopathology shows endomysial inflammatory exudates surrounding and invading nonnecr...
متن کاملSporadic inclusion body myositis: new insights and potential therapy.
PURPOSE OF THE REVIEW To describe new insights and developments in the pathogenesis, diagnosis and treatment of sporadic inclusion body myositis (IBM). RECENT FINDINGS Various hypothesis about the pathogenesis of IBM continue to be investigated, including autoimmune factors, mitochondrial dysfunction, protein dyshomeostasis, altered nucleic acid metabolism, myonuclear degeneration and the rol...
متن کاملNERVE AND MUSCLE (M HIRANO AND LH WEIMER, SECTION EDITORS) Inclusion Body Myositis
Sporadic inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy (IIM) after age 50 years. It presents with chronic insidious proximal leg and distal arm asymmetric muscle weakness. Despite similarities with polymyositis (PM), it is likely that IBM is primarily a degenerative disorder rather than inflammatory muscle disease. IBM is associated with a modest degree of cr...
متن کاملInclusion body myositis: new insights into pathogenesis.
PURPOSE OF REVIEW The pathogenesis of sporadic inclusion body myositis is complex and the disease has a relentless course. Recent observations regarding possible mechanisms of disease may provide targets for therapy. RECENT FINDINGS Evidence is strengthening that specific T-cell and B-cell responses are ongoing in skeletal muscle in sporadic inclusion body myositis and that cytokines and chem...
متن کاملSporadic inclusion body myositis: the genetic contributions to the pathogenesis
Sporadic inclusion body myositis (sIBM) is the commonest idiopathic inflammatory muscle disease in people over 50 years old. It is characterized by slowly progressive muscle weakness and atrophy, with typical pathological changes of inflammation, degeneration and mitochondrial abnormality in affected muscle fibres. The cause(s) of sIBM are still unknown, but are considered complex, with the con...
متن کامل