Inclusion body myositis: genetic factors, aberrant protein expression, and autoimmunity.
Sporadic inclusion body myositis (s-IBM) is an inflammatory myopathy mainly affecting elderly individuals. It has a chronic progressive course leading to severe disability. Immunosuppressive treatment is in most instances ineffective. S-IBM is morphologically characterized by mononuclear cell infiltrates and vacuolated muscle fibers with pathologic accumulation of a large number of different proteins. Recent research has focused on the expression of various factors that may contribute to the inflammatory reaction and the typical inclusions. This review summarizes the new information on genetic factors, abnormal protein expression and inflammation, which provides a basis for linking the different typical morphologic features of s-IBM to a cascade of pathogenic events.
Oldfors
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Göteborg Neuromuscular Center, Department of Pathology, Sahlgrenska University Hospital, Göteborg, Sweden. anders.oldfors@path.gu.se
Fyhr
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IM
D015551:Autoimmunity; D006801:Humans; D009124:Muscle Proteins / Q000378:metabolism*; D018979:Myositis, Inclusion Body / Q000235:genetics* / Q000276:immunology / Q000378:metabolism*
D009124:Muscle Proteins