Inclusion Body Myositis
نویسنده
چکیده
Inclusion body myositis is an insidious, slowly progressive myopathy of middle-aged and older individuals. Because of these characteristics, diagnosis is often delayed. Affected muscle is marked by the presence of rimmed vacuoles, inclusions, and an inflammatory infiltrate largely made up of CD8 T lymphocytes and macrophages. The inclusions contain beta-amyloid and phosphorylated tau protein, as well as other components. Theories of the pathogenesis of this disorder of older individuals include those based on evidence of the unfolded protein response leading to endoplasmic reticulum stress, abnormalities of proteosomal degradative function, mitochondrial dysfunction, the immune response, and amyloid toxicity. There is no proven, reliably effective medication for this disorder.
منابع مشابه
O 28: Autoimmune Myositis: General Aspects of Pathophysiology, Symptoms and Therapeutic Options
Idiopathic inflammatory myopathies are a heterogeneous group of muscle disorders characterized by chronic muscle inflammation and progressive muscle weakness. Polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM) are the three major subsets based on distinct clinical and histopathological features. Since the pathogenesis remains unclear, therapeutic approaches actually compr...
متن کاملInclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches.
Inclusion body myositis is the most common acquired muscle disease in older individuals, and its prevalence varies among countries and ethnic groups. The aetiology and pathogenesis of sporadic inclusion body myositis are still poorly understood; however genetic factors, ageing, and environmental triggers might all have a role. Unlike other inflammatory myopathies, sporadic inclusion body myosit...
متن کاملInclusion body myositis with granuloma formation in muscle tissue.
Inclusion body myositis is a form of inflammatory myopathy. We identified 4 cases of inclusion body myositis showing granuloma formation in muscle tissue and aimed to assess the features of this atypical form of inclusion body myositis. We retrospectively reviewed consecutive patients who satisfied European Neuromuscular Centre IBM Research Diagnostic Criteria 2011. Then, we assessed clinical p...
متن کاملA case of inclusion body myositis with benign monoclonal gammopathy successfully responding to repeated immunoabsorption.
A 69 year old woman with inclusion body myositis is described. She presented with benign monoclonal gammopathy. She was resistant to steroid therapy, but responded to repeated immunoabsorption. Up to now, there has been no established therapy for inclusion body myositis, including IVIg. It is suggested that immunoabsorption could be an alternative therapy for inclusion body myositis, when it wa...
متن کاملInclusion body myositis: a case with associated collagen vascular disease responding to treatment.
Patients with inclusion body myositis demonstrate characteristic histological and electronmicroscopical abnormalities in muscle and are generally considered refractory to treatment. A patient with inclusion body myositis is described with evidence of associated autoimmune disease, who responded to steroids.
متن کاملInclusion body myositis--a case report.
Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm t...
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