Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis

  title={Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis},
  author={Cristina Cappelletti and Lucia Ovidia Morandi and Marina Mora and Franco Salerno and Paolo Confalonieri and Renato Mantegazza and Pia Bernasconi},
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of chronic systemic disorders characterized by muscle inflammation and progressive muscle weakness. The major clinical variants are dermatomyositis (DM) including a distinct juvenile (JDM) subtype, polymyositis (PM), and inclusion body myositis (IBM) (Engel & Hohlfeld, 2004). IBM is divided into sporadic IBM (sIBM), the most common muscle disease starting after age 50 years, occurring mainly in men and leading to severe… 

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Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 50 years and probably accounts for about 30% of all inflammatory myopathies.
Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies
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Genotyping has revealed activation of the type I interferon pathway in patients with dermatomyositis or with expression of particular autoantibodies, and adaptive immunity is involved in pathogenesis, and both T and B cells are interesting targets for therapy.
Inflammatory myopathies: evaluation and management.
The clinical manifestations, pathology, and treatment approaches for the inflammatory myopathies are discussed, including inclusion body myositis and polymyositis, which are poorly understood autoimmune diseases affecting skeletal muscle.
The relative prevalence of dermatomyositis and polymyositis in Europe exhibits a latitudinal gradient
The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic diseases that include the more familiar disease entities of dermatomyositis (DM), polymyositis (PM), and inclusion
Immunotherapy of Inflammatory Myopathies: Practical Approach and Future Prospects
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Although IBM is difficult to treat, it respond to appropriate immunotherapies, if diagnosed early and treated aggressively, and emerging agents against T cells, B cells, transmigration, or transduction molecules are discussed as potential new treatment options.
Mechanisms of Disease: autoantigens as clues to the pathogenesis of myositis
A central role of autoantigens themselves as a critical partner in driving autoimmune diseases, and the potential for their therapeutic manipulation, is highlighted and insights that the cancer–autoimmunity interfaces provides into the relationship between the anticancer immune response and autoimmune diseases are highlighted.