Idiopathic Inflammatory Myopathies

B. Pathology and Pathogenesis
  • Lisa G. Rider
  • Frederick W. Miller


  • Major pathology consists of focal inhomogeneous infl ammation with injury, death, and repair of muscle cells.

  • Each subgroup of myositis has characteristic changes on microscopy and immunochemistry.

  • ■ Etiology is still unclear but selected environmental exposures in genetically predisposed hosts have been found.


Human Leucocyte Antigen Inclusion Body Myositis Endoplasmic Reticulum Stress Response Juvenile Dermatomyositis Human Leucocyte Antigen Allele 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Dalakas MC. Muscle biopsy findings in inflammatory myopathies. Rheum Dis Clin North Am 2002; 28:779–798, vi.PubMedCrossRefGoogle Scholar
  2. 2.
    Arahata K, Engel AG. Monoclonal antibody analysis of mononuclear cells in myopathies. I. Quantitation of subsets according to diagnosis and sites of accumulation and dem-onstration and counts of muscle fi bers invaded by T cells. Ann Neurol 1984;16:193–208.PubMedCrossRefGoogle Scholar
  3. 3.
    Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in child-hood polydermatomyositis. Arthritis Rheum 1982;25:126–139.PubMedCrossRefGoogle Scholar
  4. 4.
    Engel AG, Arahata K. Monoclonal antibody analysis of mononuclear cells in myopathies. II: Phenotypes of auto-invasive cells in polymyositis and inclusion body myositis. Ann Neurol 1984;16:0209–215.CrossRefGoogle Scholar
  5. 5.
    Engel AG, Arahata K. Mononuclear cells in myopathies: quantitation of functionally distinct subsets, recognition of antigen-specifi c cell-mediated cytotoxicity in some dis-eases, and implications for the pathogenesis of the differ-ent inflammatory myopathies. Hum Pathol 1986;17:704–721.PubMedCrossRefGoogle Scholar
  6. 6.
    Kissel JT, Mendell JR, Rammohan KW. Microvasculature deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986;314:329–334.PubMedCrossRefGoogle Scholar
  7. 7.
    Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol 1990;27:343–356.PubMedCrossRefGoogle Scholar
  8. 8.
    Estruch R, Grau JM, Fernandez-Sola J, Casademont J, Monforte R, Urbano-Marquez A. Microvascular changes in skeletal muscle in idiopathic inflammatory myopathy. Hum Pathol 1992;23:888–895.PubMedCrossRefGoogle Scholar
  9. 9.
    Figarella-Branger D, Civatte M, Bartoli C, Pellissier JF. Cytokines, chemokines, and cell adhesion molecules in inflammatory myopathies. Muscle Nerve 2003;28:659–682.PubMedCrossRefGoogle Scholar
  10. 10.
    Goebels N, Michaelis D, Engelhardt M, et al. Differential expression of perforin in muscle-infi ltrating T cells in polymyositis and dermatomyositis. J Clin Invest 1996; 97:2905–2910.PubMedCrossRefGoogle Scholar
  11. 11.
    O’Hanlon TP, Carrick DM, Arnett FC, et al. Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A,-B,-Cw,-DRB1 and-DQA1 allelic profi les and motifs defi ne clinicopathologic groups in Caucasians. Medicine (Baltimore) 2005;84:338–349.CrossRefGoogle Scholar
  12. 12.
    Reed AM, Ytterberg SR. Genetic and environmental risk factors for idiopathic inflammatory myopathies. Rheum Dis Clin North Am 2002;28:891–916.PubMedCrossRefGoogle Scholar
  13. 13.
    O’Hanlon TP, Carrick DM, Targoff IN, et al. HLA-A,-B,-DRB1 and-DQA1 allelic profi les for the idiopathic inflammatory myopathies: distinct immunogenetic risk and protective factors distinguish European American patients with different myositis autoantibodies. Medicine 2006;85:111–127.PubMedCrossRefGoogle Scholar
  14. 14.
    Lyon MG, Bloch DA, Hollak B, Fries JF. Predisposing factors in polymyositis-dermatomyositis: results of a nationwide survey. J Rheumatol 1989;16:1218–1224.PubMedGoogle Scholar
  15. 15.
    Dalakas MC. Mechanisms of disease. Signaling pathways and immunobiology of inflammatory myopathies. Nat Clin Pract Rheumatol 2006;2:219–227.PubMedCrossRefGoogle Scholar
  16. 16.
    Nagaraju K, Rider LG, Fan C, et al. Endothelial cell activa-tion and neovascularization are prominent in dermato-myositis. J Autoimmune Dis 2006;3:2.PubMedCrossRefGoogle Scholar
  17. 17.
    Fall N, Bove KE, Stringer K, et al. Association between lack of angiogenic response in muscle tissue and high expression of angiostatic ELR-negative CXC chemokines in patients with juvenile dermatomyositis: possible link to vasculopathy. Arthritis Rheum 2005;52:3175–3180.PubMedCrossRefGoogle Scholar
  18. 18.
    Tezak Z, Hoffman EP, Lutz JL, et al. Gene expression profi ling in DQA1*0501 children with untreated derma-tomyositis: a novel model of pathogenesis. J Immunol 2002;168:4154–4163.PubMedGoogle Scholar
  19. 19.
    Greenberg SA, Sanoudou D, Haslett JN, et al. Molecular profi les of inflammatory myopathies. Neurology 2002;59: 1170–1182.PubMedGoogle Scholar
  20. 20.
    Raju R, Dalakas MC. Gene expression profi le in the muscles of patients with inflammatory myopathies: effect of therapy with IVIG and biological validation of clini-cally relevant genes. Brain 2005;128:1887–1896.PubMedCrossRefGoogle Scholar
  21. 21.
    Nagaraju K, Raben N, Loeffler L, et al. Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specifi c autoantibodies. Proc Natl Acad Sci U S A 2000;97:9209–9214.PubMedCrossRefGoogle Scholar
  22. 22.
    Nagaraju K, Casciola-Rosen L, Lundberg I, et al. Activa-tion of the endoplasmic reticulum stress response in auto-immune myositis: potential role in muscle fiber damage and dysfunction. Arthritis Rheum 2005;52:1824–1835.PubMedCrossRefGoogle Scholar
  23. 23.
    Nogalska A, Engel WK, McFerrin J, Kokame K, Komano H, Askanas V. Homocysteine-induced endoplasmic reticulum protein (Herp) is up-regulated in sporadic inclusion-body myositis and in endoplasmic reticulum stress-induced cultured human muscle fibers. J Neurochem 2006;96: 1491–1499.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2008

Authors and Affiliations

  • Lisa G. Rider
    • 1
  • Frederick W. Miller
    • 2
  1. 1.Environmental Autoimmunity Group, Office of Clinical ResearchNational Institute of Environmental Health Sciences, National Institutes of Health, Clinical Research CenterBethesdaUSA
  2. 2.Environmental Autoimmunity Group, Office of Clinical ResearchNational Institute of Environmental Health Sciences, National Institutes of HealthBethesdaUSA

Personalised recommendations