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Article
Open AccessMulti-level profiling unravels mitochondrial dysfunction in myotonic dystrophy type 2
Myotonic dystrophy type 2 (DM2) is an autosomal-dominant multisystemic disease with a core manifestation of proximal muscle weakness, muscle atrophy, myotonia, and myalgia. The disease-causing CCTG tetranucleo...
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Article
Open AccessComplement and MHC patterns can provide the diagnostic framework for inflammatory neuromuscular diseases
Histopathological analysis stands as the gold standard for the identification and differentiation of inflammatory neuromuscular diseases. These disorders continue to constitute a diagnostic challenge due to th...
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Article
Open AccessSkeletal muscle provides the immunological micro-milieu for specific plasma cells in anti-synthetase syndrome-associated myositis
Anti-synthetase syndrome (ASyS)-associated myositis is a major subgroup of the idiopathic inflammatory myopathies (IIM) and is characterized by disease chronicity with musculoskeletal, dermatological and pulmo...
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Article
Open AccessPerformance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
There have been numerous classification systems to diagnose corresponding myositis subtypes and select appropriate therapeutic measures. However, the lack of a broad consensus on diagnostic criteria has led to...
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Article
Open AccessSystemic sclerosis-associated myositis features minimal inflammation and characteristic capillary pathology
Systemic sclerosis represents a chronic connective tissue disease featuring fibrosis, vasculopathy and autoimmunity, affecting skin, multiple internal organs, and skeletal muscles. The vasculopathy is consider...
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Article
Open AccessDifferential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis
Dermatomyositis (DM) can occur in both adults and juveniles with considerable clinical differences. The links between immune-mediated mechanisms and vasculopathy with respect to development of perifascicular p...
