Immune and myodegenerative pathomechanisms in inclusion body myositis
نویسندگان
چکیده
منابع مشابه
Immune and myodegenerative pathomechanisms in inclusion body myositis
Inclusion Body Myositis (IBM) is a relatively common acquired inflammatory myopathy in patients above 50 years of age. Pathological hallmarks of IBM are intramyofiber protein inclusions and endomysial inflammation, indicating that both myodegenerative and inflammatory mechanisms contribute to its pathogenesis. Impaired protein degradation by the autophagic machinery, which regulates innate and ...
متن کاملInclusion body myositis: advancements in diagnosis, pathomechanisms, and treatment.
PURPOSE OF REVIEW To review new advances in inclusion body myositis (IBM) and discuss them in light of current knowledge on diagnosis, pathomechanisms, and treatment perspectives. RECENT FINDINGS IBM is a treatment refractory inflammatory myopathy in middle-aged patients that leads to a slow, relentlessly progressive muscle weakness, and atrophy. Recent data collections suggest that mortality...
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Sporadic inclusion-body myositis (s-IBM), the most common muscle disease of older persons, is of unknown cause and there is no successful treatment. We summarize our most recent findings, which provide a better understanding of the steps in the pathogenetic cascade. We suggest that s-IBM is primarily a myodegenerative disease. Intriguing are the phenotypic similarities between s-IBM muscle fibe...
متن کاملSporadic inclusion body myositis - a myodegenerative disease or an inflammatory myopathy.
Sporadic inclusion body myositis (sIBM) is an insidious late-onset progressive myopathy that typically affects patients over the age of 50. Clinically, patients develop a characteristic pattern of weakness that affects the forearm flexors and knee extensors. Muscle biopsy, often utilized in the diagnosis, demonstrates a chronic myopathy with mixed pathologies harbouring intramyofiber protein in...
متن کاملInclusion-body myositis
Inclusion-body myositis is the most frequent and disabling myopathy seen in patients over 50 years of age. The distinct clinical features that lead to correct diagnosis and inclusion-body myositis mimics are highlighted. Inclusionbody myositis has a complex pathogenesis in which autoimmune and inflammatory features coexist with elements of degeneration and abundant accumulations of various stre...
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ژورنال
عنوان ژورنال: Annals of Clinical and Translational Neurology
سال: 2017
ISSN: 2328-9503
DOI: 10.1002/acn3.419