Abstract: The term idiopathic inflammatory myopathies (IIM) encompasses a heterogeneous group of muscle-dominant systemic autoimmune syndromes, including polymyositis (PM), dermatomyositis (DM), sporadic inclusion body myositis (sIBM), and immune-mediated necrotizing myopathy (IMNM). The reported incidence of IIM ranges from 5 to 10 × 10–6. Patients with PM, DM, and IMNM characteristically present with the insidious onset of symmetric proximal weakness, while in sIBM the weakness can be asymmetric, and involve the distal upper limbs and quadriceps. Dermatomyositis may also be accompanied by a range of cutaneous manifestations. Raised serum creatine kinase levels, the presence of characteristic myositis-specific antibodies, myopathic triad on electromyography, and myoedema on muscle magnetic resonance imaging are helpful in supporting a diagnosis of IIM. Muscle biopsy is the definitive diagnostic test and serves to distinguish subsets of disease, which each have characteristic histopathological changes reflecting underlying differences in pathogenesis. Mortality remains elevated in patients with IIM, despite the advent of immunosuppressive therapies.
Publication Year: 2018
Publication Date: 2018-01-01
Language: en
Type: book
Indexed In: ['crossref']
Access and Citation
AI Researcher Chatbot
Get quick answers to your questions about the article from our AI researcher chatbot