Idiopathic Inflammatory Myopathies

  • Robert L. Wortmann


The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of disorders characterized by symmetric proximal muscle weakness and elevated serum levels of enzymes derived from skeletal muscle. These include creatine phosphokinase (CPK), aldolase, aspartate, and alanine aminotransferases (AST and ALT), and lactate dehydrogenase (LDH). In addition, electromyography (EMG), magnetic resonance imaging (MRI), and muscle histology show changes indicative of nonsupparative inflammation (Table 18A-1) (1).


Creatine Kinase Interstitial Lung Disease Human Leucocyte Antigen Inclusion Body Myositis Idiopathic Inflammatory Myopathy 
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.


  1. 1.
    Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344–347, 403–407.CrossRefPubMedGoogle Scholar
  2. 2.
    Lindsley CB. Juvenile dermatomyositis update. Curr Opin Rheumatol 2006;8:174–177.Google Scholar
  3. 3.
    Targoff IN. Myositis specific autoantibodies. Curr Opin Rheumatol 2006;8:196–206.Google Scholar
  4. 4.
    Santmyire-Rosenberger B, Dugan EM. Skin involvement in dermatomyositis. Curr Opin Rheumatol 2003;15:714–722.CrossRefPubMedGoogle Scholar
  5. 5.
    Kang EH, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology 2005;44:1282–1286.CrossRefPubMedGoogle Scholar
  6. 6.
    Schnabel A, Hellmich B, Gross WL. Interstitial lung disease in polymyositis and dermatomyositis. Curr Rheum Rep 2005;7:99–105.CrossRefGoogle Scholar
  7. 7.
    Bonnefoy O, et al. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol 2004;49:235–244.CrossRefPubMedGoogle Scholar
  8. 8.
    Yazici Y, Kagen LJ. Cardiac involvement in myositis. Curr Opin Rheumatol 2002;14:663–665.CrossRefPubMedGoogle Scholar
  9. 9.
    Buchbinder R, Hill CL. Malignancy in patients with inflammatory myopathy. Curr Rheum Rep 2002;4:415–426.CrossRefGoogle Scholar
  10. 10.
    Whitmore SE, et al. Dermatomyositis sine myositis: association with malignancy. J Rheumatol 1996;23:101–105.PubMedGoogle Scholar
  11. 11.
    Chen Y-J, Wu C-Y, Shen J-L. Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol 2001;144:825–831.CrossRefPubMedGoogle Scholar
  12. 12.
    Sultan SM. Clinical assessment in adult onset idiopathic inflammatory myopathy. Curr Opin Rheumatol 2004;16:668–672.CrossRefPubMedGoogle Scholar
  13. 13.
    Targoff I. Laboratory testing in the diagnosis and management of idiopathic inflammatory myopathies. Rheum Dis Clin North Am 2002;28:859–890.CrossRefPubMedGoogle Scholar
  14. 14.
    Grundtman C, Lundberg IE. Pathogenesis of idiopathic inflammatory myopathies. Curr Opin Rheumatol 2006;8:188–195.Google Scholar
  15. 15.
    Scott DL, Kingsley GH. Use of imaging to assess patients with muscle disease. Curr Opin Rheumatol 2004;16:678–683.CrossRefPubMedGoogle Scholar
  16. 16.
    Arakawa H, et al. Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation. Chest 2003;123:1096–1103.CrossRefPubMedGoogle Scholar
  17. 17.
    Tansey D, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 2004;44:585–596.CrossRefPubMedGoogle Scholar
  18. 18.
    Marton K, et al. Evaluation of oral manifestations and masticatory force in patients with polymyositis and dermatomyositis. J Oral Pathol Med 2005;34:164–169.CrossRefPubMedGoogle Scholar
  19. 19.
    Ponyi A, et al. Disease course, frequency of relapses and survival of 73 patients with juvenile or adult dermatomyositis. Clin Exp Rheum 2005;23:50–56.Google Scholar
  20. 20.
    Ponyi A, et al. Functional outcome and quality of life in adult patients with idiopathic inflammatory myositis. Rheumatology 2005;44:83–88.CrossRefPubMedGoogle Scholar
  21. 1.
    Dalakas MC. Muscle biopsy findings in inflammatory myopathies. Rheum Dis Clin North Am 2002;28:779–798, vi.CrossRefPubMedGoogle Scholar
  22. 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 demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984;16:193–208.CrossRefPubMedGoogle Scholar
  23. 3.
    Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum 1982;25:126–139.CrossRefPubMedGoogle Scholar
  24. 4.
    Engel AG, Arahata K. Monoclonal antibody analysis of mononuclear cells in myopathies. II: Phenotypes of autoinvasive cells in polymyositis and inclusion body myositis. Ann Neurol 1984;16:209–215.CrossRefPubMedGoogle Scholar
  25. 5.
    Engel AG, Arahata K. Mononuclear cells in myopathies: quantitation of functionally distinct subsets, recognition of antigen-specific cell-mediated cytotoxicity in some diseases, and implications for the pathogenesis of the different inflammatory myopathies. Hum Pathol 1986;17:704–721.CrossRefPubMedGoogle Scholar
  26. 6.
    Kissel JT, Mendell JR, Rammohan KW. Microvasculature deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986;314:329–334.CrossRefPubMedGoogle Scholar
  27. 7.
    Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol 1990;27:343–356.CrossRefPubMedGoogle Scholar
  28. 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.CrossRefPubMedGoogle Scholar
  29. 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.CrossRefPubMedGoogle Scholar
  30. 10.
    Goebels N, Michaelis D, Engelhardt M, et al. Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis. J Clin Invest 1996;97:2905–2910.CrossRefPubMedGoogle Scholar
  31. 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 profiles and motifs define clinicopathologic groups in Caucasians. Medicine (Baltimore) 2005;84:338–349.CrossRefGoogle Scholar
  32. 12.
    Reed AM, Ytterberg SR. Genetic and environmental risk factors for idiopathic inflammatory myopathies. Rheum Dis Clin North Am 2002;28:891–916.CrossRefPubMedGoogle Scholar
  33. 13.
    O’Hanlon TP, Carrick DM, Targoff IN, et al. HLA-A, -B, -DRB1 and -DQA1 allelic profiles for the idiopathic inflammatory myopathies: distinct immunogenetic risk and protective factors distinguish European American patients with different myositis autoantibodies. Medicine 2006;85:111–127.CrossRefPubMedGoogle Scholar
  34. 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
  35. 15.
    Dalakas MC. Mechanisms of disease. Signaling pathways and immunobiology of inflammatory myopathies. Nat Clin Pract Rheumatol 2006;2:219–227.CrossRefPubMedGoogle Scholar
  36. 16.
    Nagaraju K, Rider LG, Fan C, et al. Endothelial cell activation and neovascularization are prominent in dermatomyositis. J Autoimmune Dis 2006;3:2.CrossRefPubMedGoogle Scholar
  37. 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.CrossRefPubMedGoogle Scholar
  38. 18.
    Tezak Z, Hoffman EP, Lutz JL, et al. Gene expression profiling in DQA1*0501+ children with untreated dermatomyositis: a novel model of pathogenesis. J Immunol 2002;168:4154–4163.PubMedGoogle Scholar
  39. 19.
    Greenberg SA, Sanoudou D, Haslett JN, et al. Molecular profiles of inflammatory myopathies. Neurology 2002;59:1170–1182.PubMedGoogle Scholar
  40. 20.
    Raju R, Dalakas MC. Gene expression profile in the muscles of patients with inflammatory myopathies: effect of therapy with IVIG and biological validation of clinically relevant genes. Brain 2005;128:1887–1896.CrossRefPubMedGoogle Scholar
  41. 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-specific autoantibodies. Proc Natl Acad Sci U S A 2000;97:9209–9214.CrossRefPubMedGoogle Scholar
  42. 22.
    Nagaraju K, Casciola-Rosen L, Lundberg I, et al. Activation of the endoplasmic reticulum stress response in autoimmune myositis: potential role in muscle fiber damage and dysfunction. Arthritis Rheum 2005;52:1824–1835.CrossRefPubMedGoogle Scholar
  43. 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.CrossRefPubMedGoogle Scholar
  44. 1.
    Miller FW, Rider LG, Chung YL, et al. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology 2001;40:1262–1273.CrossRefPubMedGoogle Scholar
  45. 2.
    Rider LG, Giannini EH, Brunner HI, et al. International consensus on preliminary definitions of improvement in adult and juvenile myositis. Arthritis Rheum 2004;50:2281–2290.CrossRefPubMedGoogle Scholar
  46. 3.
    Oddis CV, Rider LG, Reed AM, et al. International consensus guidelines for trials of therapies in the idiopathic inflammatory myopathies. Arthritis Rheum 2005;52:2607–2615.CrossRefPubMedGoogle Scholar
  47. 4.
    Guzman J, Petty RE, Malleson PN. Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes. J. Rheumatol 1994;21:739–743.PubMedGoogle Scholar
  48. 5.
    Johnston JD, Lloyd M, Mathews JA, et al. Racial variation in serum creatine kinase levels. J Roy Soc Med 1996;89:462–464.PubMedGoogle Scholar
  49. 6.
    Scott DL, Kingsley GH. Use of imaging to assess patients with muscle disease. Curr Opin Rheumatol 2004;16:678–683.CrossRefPubMedGoogle Scholar
  50. 7.
    Dion E, Cherin P, Payan C, et al. Magnetic resonance imaging criteria for distinguishing between inclusion body myositis and polymyositis. J Rheumatol 2002;29:1897–1906.PubMedGoogle Scholar
  51. 8.
    Schnabel A, Hellmich B, Gross WL. Interstitial lung disease in polymyositis and dermatomyositis. Curr Rheum Rep 2005;7:99–105.CrossRefGoogle Scholar
  52. 9.
    Targoff IN. Idiopathic inflammatory myopathy: autoantibody update. Curr Rheum Rep 2002;4:434–441.CrossRefGoogle Scholar
  53. 10.
    Brouwer R, Hengstman GJ, Egberts WT, et al. Autoantibody profiles in the sera of European patients with myositis. Ann Rheum Dis 2001;60:116–123.CrossRefPubMedGoogle Scholar
  54. 11.
    Kao AH, Lacomis DH, Lucas M, et al. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum 2004;50:209–215.CrossRefPubMedGoogle Scholar
  55. 12.
    Hengstman GJ, Brouwer R, Egberts WT, et al. Clinical and serological characteristics of 125 Dutch myositis patients. Myositis-specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies. J Neurol 2002;249:69–75.CrossRefPubMedGoogle Scholar
  56. 13.
    Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 2001;357:96–100.CrossRefPubMedGoogle Scholar
  57. 14.
    Baer AN. Advances in the therapy of idiopathic inflammatory myopathies. Curr Opin Rheumatol 2006;18:236–241.CrossRefPubMedGoogle Scholar
  58. 15.
    Villalba L, Hick JE, Adams EM, et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 1998;41:392–399.CrossRefPubMedGoogle Scholar
  59. 16.
    Wilkes MR, Sereika SM, Fertig N, et al. Treatment of antisynthetase-associated interstitial lung disease with tacrolimus. Arthritis Rheum 2005;52:2439–2446.CrossRefPubMedGoogle Scholar
  60. 17.
    Barohn RJ, Amato AA, Sahenk Z, et al. Inclusion body myositis: explanation for poor response to immunosuppressive therapy. Neurology 1995;45:1302–1304.PubMedGoogle Scholar
  61. 18.
    Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993;329:1993–2000.CrossRefPubMedGoogle Scholar
  62. 19.
    Levine TD. Rituximab in the treatment of dermatomy-ositis: an open-label pilot study. Arthritis Rheum 2005;52:601–607.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Robert L. Wortmann
    • 1
  1. 1.Department of Internal MedicineThe University of Oklahoma College of MedicineTulsaUSA

Personalised recommendations