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Abstract

The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of disorders characterized by varied patterns of inflammation within striated muscle. The major disease categories among the IIM are der-matomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), myositis associated with connective tissue diseases, and myositis associated with malignancy. The skin, joints, lungs, heart, and gastrointestinal tract are also involved in different forms of these disorders. Muscle weakness that is proximal, symmetrical, and painless is a hallmark feature of the inflammatory myopathies. Patients with IBM are also prone to distal, asymmetric muscle involvement. Measurement of serum concentrations of muscle enzymes, skin and muscle biopsy, electromyography, and magnetic resonance imaging can assist in the diagnosis. Testing for serum autoantibodies is helpful both in diagnosis and in predicting the clinical phenotype and response to therapy. A minority of patients with DM and PM have myositis that is associated with an underlying malignancy. The risk is greatest for middle-aged to elderly patients with DM. Approximately 15% of DM patients have malignancy-associated disease. Most malignancies present within 1 year before or after the diagnosis of inflammatory myopathy.

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References

  • Alexanderson H, Dastalmachi M, Esbjornsson-Liljedahl M, et al Benefits of intensive resistance training in patients with chronic polymyositis or dermatomyositis. Arthritis Care Res 2007; 57:768.

    Article  Google Scholar 

  • Bohan A, Peter JB. Polymyositis and dermatomyositis. Parts 1 and 2 N. Engl J Med 1975; 292:344–3447,403–407

    Article  PubMed  CAS  Google Scholar 

  • Buchbinder R, Forbes A, Hall S, Dennett X, Giles G. Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study. Ann Intern Med 2001; 134:1087–1095.

    CAS  Google Scholar 

  • Callen JP. Relation between dermatomyositis and polymyositis and cancer. Lancet 2001; 357:85–86

    Article  PubMed  CAS  Google Scholar 

  • Chinoy H, Fertig N, Oddis C V, et al The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis. Ann Rheum Dis 2007; 66:1345–1349

    Article  PubMed  Google Scholar 

  • Dimitri D, Andre C, Roucoules J, et al Myopathy associated with anti-signal recognition peptide antibodies: Alinical heterogeneity contrasts with stereotyped histopathology. Muscle Nerve 2007; 35:389–395

    Article  PubMed  Google Scholar 

  • Dion E, Cherin P, Payan C, et al Magnetic resonance imaging criteria for distinguishing between inclusion body myositis and polymyosi-tis. J Rheumatol. 2002; 29:1897

    PubMed  Google Scholar 

  • Erlacher P, Lercher A, Falkensammer J, et al Cardiac troponin and beta-type myosin heavy chain concentrations in patients with polymyosi-tis or dermatomyositis. Clin Chim Acta 2001; 306:27–33

    Article  PubMed  CAS  Google Scholar 

  • Friedman AW, Targoff IN, Arnett FC. Interstitial lung disease with autoan-tibodies against aminoacyl- tRNA synthetases in the absence of clinically apparent myositis. Semin Arthritis Rheum 1996; 26:459–467

    Article  PubMed  CAS  Google Scholar 

  • Gerami P, Schope JM, McDonald L, et al A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): A missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 2006; 54:597–613

    Article  PubMed  Google Scholar 

  • Hengstman GJ, van Brenk L, Vree Egberts WT, et al High specificity of myositis specific autoantibodies for myositis compared with other neuromuscular disorders. J Neurol 2005; 252:534–537

    Article  PubMed  CAS  Google Scholar 

  • Hengstman GJ, ter Laak HJ, Vree Egberts WT, et al Anti-signal recognition particle autoantibodies: Marker of a necrotising myopathy. Ann Rheum Dis 2006; 65:1635–1638

    Article  PubMed  CAS  Google Scholar 

  • Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, Evans SR, Felson DT. Frequency of specific cancer types in der-matomyositis and polymyositis: a population-based study. Lancet 2001; 357:96–100

    Article  PubMed  CAS  Google Scholar 

  • Hirakata M, Suwa A, Takada T, et al Clinical and immunogenetic features of patients with autoantibodies to asparaginyl-transfer RNA synthetase. Arthritis Rheum 2007; 56:1295–1303

    Article  PubMed  CAS  Google Scholar 

  • Jablonska S, Blaszczyk M. Scleroderma overlap syndromes. Adv Exp Med Biol 1999; 455:85–92

    Article  PubMed  CAS  Google Scholar 

  • Kagen LJ, Aram S. Creatine kinase activity inhibitor in sera from patients with muscle disease. Arthritis Rheum 1987; 30: 213–217

    Article  PubMed  CAS  Google Scholar 

  • Kaji K, Fujimoto M, Hasegawa M, et al Identification of a novel autoanti-body reactive with 155 and 140 kDa nuclear proteins in patients with dermatomyositis: An association with malignancy. Rheumatology 2007; 6:25–28

    Article  PubMed  CAS  Google Scholar 

  • Kao AH, Lacomis D, Lucas M, et al Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflam-matory myopathy. 2004; Arthritis Rheum. 50:209–215

    Article  PubMed  CAS  Google Scholar 

  • Koenig M, Fritzler MJ, Targoff IN, et al Heterogeneity of autoantibod-ies in 100 patients with autoimmune myositis: Insights into clinical features and outcomes. Arthritis Res Therapy 2007; 9:R78

    Article  Google Scholar 

  • Love LA, Leff RL, Fraser DD, et al A new approach to the classification of idiopathic inflammatory myopathy: Myositis-specific autoanti-bodies define useful homogeneous patient groups. Medicine 1991; 70:360–374

    Article  PubMed  CAS  Google Scholar 

  • Miller T, Al Lozi MT, Lopate G, et al Myopathy with antibodies to the signal recognition particle: Clinical and pathological features. J Neurol Neurosurg Psychiatry 2002; n73–420 428

    Article  PubMed  CAS  Google Scholar 

  • Oddis C V, Medsger TA Jr., Cooperstein LA. A subluxing arthropathy associated with the anti-Jo-1 antibody in polymyositis/dermatomy-ositis. Arthritis Rheum 1990; 33:1640–1645

    Article  PubMed  CAS  Google Scholar 

  • Oddis CV, Okano Y, Rudert WA, et al Serum autoantibody to the nucle-olar antigen PM-Scl: clinical and immunogenetic associations. Arthritis Rheum 1992; 35:1211–1217

    Article  PubMed  CAS  Google Scholar 

  • Oh TH, Brumfield KA, Hoskin TL, et al Dysphagia in inflammatory myopathy: Clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007; 82: 441–447

    Article  PubMed  Google Scholar 

  • Quain RD, Teal V, Taylor L, et al Number, characteristics, & classification of dermatomyositis patients seen by dermatology & rheumatology at a tertiary medical center. J Am Acad Dermatol 2007; 57:937–943

    Article  Google Scholar 

  • Rider LG, Giannini EH, Brunner HI, et al International consensus on preliminary definitions of improvement in adult and juvenile myosi-tis. Arthritis Rheum 2004; 50:2281–2290

    Article  PubMed  Google Scholar 

  • Sato S, Kuwana M, Hirakata M. Clinical characteristics of Japanese patients with anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibod-ies. Rheumatology 2007; 46:842–845

    Article  PubMed  CAS  Google Scholar 

  • Schmidt WA, Wetzel W, Friedlander R, et al Clinical and serological aspects of patients with anti-Jo-1 antibodies–an evolving spectrum of disease manifestations. Clin Rheumatol 2000; 19:371–377

    Article  PubMed  CAS  Google Scholar 

  • Targoff IN, Arnett FC. Clinical manifestations in patients with antibody to PL-12 antigen (alanyl-tRNA synthetase). Am J Med 1990; 88: 241–251

    Article  PubMed  CAS  Google Scholar 

  • Targoff IN, Trieu EP, Miller FW. Reaction of anti-OJ autoantibodies with components of the multi-enzyme complex of aminoacyl-tRNA synthetases in addition to isoleucyl-tRNA synthetase. J Clin Invest 1993; 91:2556–2564

    Article  PubMed  CAS  Google Scholar 

  • Targoff IN, Mamyrova G, Trieu EP, et al A novel autoantibody to a 155-kd protein is associated with dermatomyositis Arthritis Rheum 2006; 54:3682–3689

    Article  PubMed  CAS  Google Scholar 

  • Tillie-Leblond I, Wislez M, Valeyre D, et al Interstitial lung disease and anti-Jo-1 antibodies: Difference between acute and gradual onset. Thorax 2008; 63:53–59

    Article  PubMed  CAS  Google Scholar 

  • Villalba L, Hicks J, Adams E, et al Treatment of refractory myositis: A randomized crossover study of two new cytoxic regimens. Arthritis Rheum 1998; 41:392–399

    Article  PubMed  CAS  Google Scholar 

  • Whitcomb ME, Schwarz MI, Tormey DC. Methotrexate pneumonitis: Case report and review of the literature. Thorax 1972; 27(5):636–639

    Article  PubMed  CAS  Google Scholar 

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Targoff, I.N., Oddis, C.V., Plotz, P.H., Miller, F.W., Gourley, M. (2009). Inflammatory Myopathies. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_18

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  • DOI: https://doi.org/10.1007/978-1-84800-934-9_18

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