Skip to main content
SpringerLink
Log in

Myositiden

Was ist der aktuelle Stand?

Myositides

What is the current situation?

  • Leitthema
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Dieser Beitrag gibt eine Übersicht über Einteilung, Diagnostik und Therapie der entzündlichen Muskelkrankheiten aus neurologischer Sicht. Die Myositissyndrome lassen sich grundsätzlich in 4 Gruppen einteilen: Polymyositis (PM), Dermatomyositis (DM), Einschlusskörperchenmyositis (IBM) und die in den letzten Jahren als neue Entität beschriebene nekrotisierende Myopathie (NM). Diese Krankheitsbilder unterscheiden sich in wesentlichen klinischen und histologischen Aspekten. Daneben gibt es Myositiden im Rahmen autoimmuner Systemerkrankungen („Overlap-Syndrom“). Die Diagnostik der Myositissyndrome basiert auf den klinischen Symptomen, der Bestimmung der Kreatin-Phosphokinase und Akutphasen-Parameter im Blut (CRP, BSR), der Antikörperdiagnostik, der Elektromyographie sowie dem MRT-Befund der Muskulatur. Die Muskelbiopsie ist zur Diagnosestellung unerlässlich. Qualitativ gute kontrollierte Studien zur Behandlung der myositischen Syndrome fehlen. Aufgrund empirischer Erfahrung ist am Nutzen einer immunsuppressiven Therapie bei PM, DM und NM aber nicht zu zweifeln.

Abstract

This article gives a review of the classification, diagnostic procedures and treatment of idiopathic inflammatory myopathies from a neurological point of view. The myositis syndromes can be subdivided into four groups, polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and necrotizing myopathy (NM), which substantially differ clinically and pathophysiologically. Myositis may also occur in association with cancer or autoimmune systemic diseases (overlap syndrome). Diagnosis of inflammatory myopathies is based on clinical symptoms, determination of creatine phosphokinase and acute phase parameters in blood (e.g. C-reactive protein and erythrocyte sedimentation rate), electromyography results and findings of magnetic resonance imaging (MRI) in muscle. A muscle biopsy is mandatory to confirm the diagnosis. High quality randomized controlled trials of treatment regimens for inflammatory myopathies are sparse; however, empirical experience indicates a clear effectiveness of immunosuppressive treatment of PM, DM and NM.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6
Abb. 7

Literatur

  1. Amato AA, Barohn RJ (2009) Evaluation and treatment of inflammatory myopathies. J Neurol Neurosurg Psychiatry 80:1060–1068

    Article  CAS  PubMed  Google Scholar 

  2. Amato AA, Greenberg SA (2013) Inflammatory myopathies. Continuum (Minneap Minn) 19:1615–1633

  3. Amato AA, Griggs RC (2003) Treatment of idiopathic inflammatory myopathies. Curr Opin Neurol 16:569–575

    Article  CAS  PubMed  Google Scholar 

  4. Amato AA, Sivakumar K, Goyal N et al (2014) Treatment of sporadic inclusion body myositis with bimagrumab. Neurology 83:2239–2246

    Article  CAS  PubMed  Google Scholar 

  5. András C, Ponyi A, Constantin T et al (2008) Dermatomyositis and polymyositis associated with malignancy: a 21-year retrospective study. J Rheumatol 35:438–444

    PubMed  Google Scholar 

  6. Azzabou N, Loureiro de Sousa P, Caldas E, Carlier PG (2015) Validation of a generic approach to muscle water T2 determination at 3T in fat-infiltrated skeletal muscle. J Magn Reson Imaging 41:645–653

    Article  PubMed  Google Scholar 

  7. Baudin PY, Azzabou N, Carlier PG, Paragios N (2012) Prior knowledge, random walks and human skeletal muscle segmentation. Med Image Comput Comput Assist Interv 15:569–576

    CAS  PubMed  Google Scholar 

  8. De Bleecker JL, Lundberg IE, De Visser M (2013) 193rd ENMC International workshop pathology diagnosis of idiopathic inflammatory myopathies 30 November – 2 December 2012, Naarden, The Netherlands. Neuromuscul Disord 23:945–951

    Article  Google Scholar 

  9. Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344–347

    Article  CAS  PubMed  Google Scholar 

  10. Brady S, Squier W, Hilton-Jones D (2013) Clinical assessment determines the diagnosis of inclusion body myositis independently of pathological features. J Neurol Neurosurg Psychiatry 84:1240–1246

    Article  PubMed  Google Scholar 

  11. Buchthal F, Pinelli P (1953) Muscle action potentials in polymyositis. Neurology 3:424–436

    Article  CAS  PubMed  Google Scholar 

  12. Bunch TW (1981) Prednisone and azathioprine for polymyositis: long-term followup. Arthritis Rheum 24:45–48

    Article  CAS  PubMed  Google Scholar 

  13. Bunch TW, Worthington JW, Combs JJ et al (1980) Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med 92:365–369

    Article  CAS  PubMed  Google Scholar 

  14. Caramaschi P, Volpe A, Carletto A et al (2007) Long-standing refractory polymyositis responding to mycophenolate mofetil: a case report and review of the literature. Clin Rheumatol 26:1795–1796

    Article  PubMed  Google Scholar 

  15. Carlier PG (2014) Global T2 versus water T2 in NMR imaging of fatty infiltrated muscles: Different methodology, different information and different implications. Neuromuscul Disord 24:390–392

    Article  PubMed  Google Scholar 

  16. Chahin N, Engel AG (2008) Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology 70:418–424

    Article  PubMed  Google Scholar 

  17. Cherin P, Pelletier S, Teixeira A et al (2002) Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: An open study with thirty-five adult patients. Arthritis Rheum 46:467–474

    Article  CAS  PubMed  Google Scholar 

  18. Couderc M, Gottenberg JE, Mariette X et al (2011) Efficacy and safety of rituximab in the treatment of refractory inflammatory myopathies in adults: results from the AIR registry. Rheumatology 50:2283–2289

    Article  CAS  PubMed  Google Scholar 

  19. Dalakas MC, Illa I, Dambrosia JM et al (1993) A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 329:1993–2000

    Article  CAS  PubMed  Google Scholar 

  20. Dalakas MC, Rakocevic G, Schmidt J et al (2009) Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion-body myositis. Brain 132:1536–1544

    Article  PubMed Central  PubMed  Google Scholar 

  21. DeVere R, Bradley WG (1975) Polymyositis: its presentation, morbidity and mortality. Brain 98:637–666

    Article  CAS  PubMed  Google Scholar 

  22. Diot E, Carmier D, Marquette D et al (2011) IV immunoglobulin might be considered as a first-line treatment of severe interstitial lung disease associated with polymyositis. Chest 140:562–563

    Article  PubMed  Google Scholar 

  23. Dubourg O, Wanschitz J, Maisonobe T et al (2011) Diagnostic value of markers of muscle degeneration in sporadic inclusion body myositis. Acta Myol 30:103–108

    CAS  PubMed Central  PubMed  Google Scholar 

  24. Frikha F, Rigolet A, Behin A et al (2009) Efficacy of rituximab in refractory and relapsing myositis with anti-JO1 antibodies: a report of two cases. Rheumatology 48:1166–1168

    Article  CAS  PubMed  Google Scholar 

  25. Gerami P, Walling HW, Lewis J et al (2007) A systematic review of juvenile-onset clinically amyopathic dermatomyositis. Br J Dermatol 157:637–644

    Article  CAS  PubMed  Google Scholar 

  26. Gordon PA, Winer JB, Hoogendijk JE, Choy EHS (2012) Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev (4):CD003643 (PMID: 19821312). Cochrane Database Syst Rev 8:CD003643

    PubMed  Google Scholar 

  27. Grable-Esposito P, Katzberg HD, Greenberg SA et al (2010) Immune-mediated necrotizing myopathy associated with statins. Muscle Nerve 41:185–190

    CAS  PubMed  Google Scholar 

  28. Del Grande F, Carrino JA, Del Grande M et al (2011) Magnetic resonance imaging of inflammatory myopathies. Top Magn Reson Imaging 22:39–43

    Article  Google Scholar 

  29. Griggs RC, Askanas V, DiMauro S et al (1995) Inclusion body myositis and myopathies. Ann Neurol 38:705–713

    Article  CAS  PubMed  Google Scholar 

  30. Hayter SM, Cook MC (2012) Updated assessment of the prevalence, spectrum and case definition of autoimmune disease. Autoimmun Rev 11:754–765

    Article  PubMed  Google Scholar 

  31. Hengstman GJD, Engelen BGM van, Venrooij WJ van (2004) Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations. Curr Opin Rheumatol 16:692–699

    PubMed  Google Scholar 

  32. Janiczek RL, Gambarota G, Sinclair CDJ et al (2011) Simultaneous T2 and lipid quantitation using IDEAL-CPMG. Magn Reson Med 66:1293–1302

    Article  PubMed  Google Scholar 

  33. Jordan B, Hanisch F, Zierz S (2011) Myositiden. Nervenheilkunde 10:776–786

    Google Scholar 

  34. Kagan LJ (1977) Myoglobinemia in inflammatory myopathies. JAMA 237:1448–1452

    Article  CAS  PubMed  Google Scholar 

  35. Lambert EH, Sayre GP, Eaton LM (1954) Electrical activity of muscle in polymyositis. Trans Am Neurol Assoc 13:64–69

    CAS  PubMed  Google Scholar 

  36. Love LA, Leff RL, Fraser DD et al (1991) A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 70:360–374

  37. Mammen AL, Chung T, Christopher-Stine L et al (2011) Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme a reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum 63:713–721

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  38. Marie I, Menard J-F, Hatron PY et al (2010) Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients. Arthritis Care Res (Hoboken) 62:1748–1755

    Article  Google Scholar 

  39. Mechler F (1974) Changing electromyographic findings during the chronic course of polymyositis. J Neurol Sci 23:237–242

    Article  CAS  PubMed  Google Scholar 

  40. Mercuri E, Pichiecchio A, Allsop J et al (2007) Muscle MRI in inherited neuromuscular disorders: Past, present, and future. J Magn Reson Imaging 25:433–440

    Article  PubMed  Google Scholar 

  41. Van der Meulen MFG, Bronner IM, Hoogendijk JE et al (2003) Polymyositis: an overdiagnosed entity. Neurology 61:316–321

    Article  Google Scholar 

  42. Miller J, Walsh Y, Saminaden S et al (2002) Randomised double-blind controlled trial of methotrexate and steroids compared with azathioprine and steroids in the treatment of idiopathic inflammatory myopathy. J Neurol Sci 199:S53

    Google Scholar 

  43. Mohassel P, Mammen AL (2013) Statin-associated autoimmune myopathy and anti-HMGCR autoantibodies. Muscle Nerve 48:477–483

    Article  CAS  PubMed  Google Scholar 

  44. Needham M, Fabian V, Knezevic W et al (2007) Progressive myopathy with up-regulation of MHC-I associated with statin therapy. Neuromuscul Disord 17:194–200

    Article  PubMed  Google Scholar 

  45. Nirkko AC, Rösler KM, Hess CW (1995) Sensitivity and specificity of needle electromyography: a prospective study comparing automated interference pattern analysis with single motor unit potential analysis. Electroencephalogr Clin Neurophysiol 97:1–10

    Article  CAS  PubMed  Google Scholar 

  46. Oddis CV, Reed AM, Aggarwal R et al (2013) Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 65:314–324

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  47. Payan J (1984) Electromyography in polymyositis and some related disorders. Clin Rheum Dis 10:75–83

    CAS  PubMed  Google Scholar 

  48. Ramanan AV, Campbell-Webster N, Ota S et al (2005) The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum 52:3570–3578

    Article  CAS  PubMed  Google Scholar 

  49. Riddoch D, Morgan-Hughes JA (1975) Prognosis in adult polymyositis. J Neurol Sci 26:71–80

    Article  CAS  PubMed  Google Scholar 

  50. Rose MR (2013) 188th ENMC International Workshop: Inclusion Body Myositis, 2–4 December 2011, Naarden, The Netherlands. Neuromuscul Disord 23:1044–1055

    Article  PubMed  Google Scholar 

  51. Rowin J, Amato AA, Deisher N et al (2006) Mycophenolate mofetil in dermatomyositis: is it safe? Neurology 66:1245–1247

    Article  CAS  PubMed  Google Scholar 

  52. Schmidt J (2009) Pathogenese der Einschlusskörperchenmyositis: Stellenwert der Entzündung und Bedeutung für Therapiestrategien. Aktuelle Neurol 36:121–125

    Article  Google Scholar 

  53. Schmidt J, Vorgerd M (2011) Therapiestandard bei Myositiden und Muskeldystrophien. Nervenarzt 82:723–732

    Article  CAS  PubMed  Google Scholar 

  54. Schoser B (2009) Inflammatory myopathies. Z Rheumatol 68:665–675 (quiz 676–677)

    Article  CAS  PubMed  Google Scholar 

  55. Sem M, Molber Ø, Lund MB, Gran JT (2009) Rituximab treatment of the anti-synthetase syndrome – a retrospective case series. Rheumatology 48:968–971

    Article  CAS  PubMed  Google Scholar 

  56. Seshadri R, Feldman BM, Ilowite N et al (2008) The role of aggressive corticosteroid therapy in patients with juvenile dermatomyositis: a propensity score analysis. Arthritis Rheum 59:989–995

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  57. Streib EW, Wilbourn AJ, Mitsumoto H (1979) Spontaneous electrical muscle fiber activity in polymyositis and dermatomyositis. Muscle Nerve 2:14–18

    Article  CAS  PubMed  Google Scholar 

  58. Thibaud J-L, Azzabou N, Barthelemy I et al (2012) Comprehensive longitudinal characterization of canine muscular dystrophy by serial NMR imaging of GRMD dogs. Neuromuscul Disord 22(Suppl 2):S85–S99

    Article  PubMed  Google Scholar 

  59. Tournadre A (2014) Prise en charge thérapeutique des polymyosites, dermatomyosites, myosites de chevauchement et myopathies nécrosantes auto-immunes. Rev Med Interne 35:466–471

    Article  CAS  PubMed  Google Scholar 

  60. Troyanov Y, Targoff IN, Tremblay J-L et al (2005) Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine (Baltimore) 84:231–249

  61. Uchino M, Yamashita S, Uchino K et al (2012) Long-term outcome of polymyositis treated with high single-dose alternate-day prednisolone therapy. Eur Neurol 68:117–121

    Article  CAS  PubMed  Google Scholar 

  62. Uncini A, Lange DJ, Lovelace RE et al (1990) Long-duration polyphasic motor unit potentials in myopathies: a quantitative study with pathological correlation. Muscle Nerve 13:263–267

    Article  CAS  PubMed  Google Scholar 

  63. Vencovský J, Jarosová K, Machácek S et al (2000) Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis. Scand J Rheumatol 29:95–102

    Article  PubMed  Google Scholar 

  64. Villalba L, Hicks JE, Adams EM et al (1998) Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 41:392–399

    Article  CAS  PubMed  Google Scholar 

  65. Van de Vlekkert J, Hoogendijk JE, Haan RJ de et al (2010) Oral dexamethasone pulse therapy versus daily prednisolone in sub-acute onset myositis, a randomised clinical trial. Neuromuscul Disord 20:382–389

    Article  Google Scholar 

  66. Vlekkert J Van De, Maas M, Hoogendijk JE et al (2015) Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic inflammatory myopathy Combining MRI and muscle biopsy improves diagnostic accuracy in subacute-onset idiopathic inflammatory myopathy. Muscle Nerve 51:253–258

    Article  Google Scholar 

  67. Werner JL, Christopher-Stine L, Ghazarian SR et al (2012) Antibody levels correlate with creatine kinase levels and strength in anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Arthritis Rheum 64:4087–4093

    Article  CAS  PubMed Central  PubMed  Google Scholar 

Download references

Einhaltung ethischer Richtlinien

Interessenkonflikt. K.M. Rösler und O. Scheidegger geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Author information

Authors and Affiliations

  1. Neuromuskuläres Zentrum, Neurologische Universitätsklinik, Inselspital, 3010, Bern, Schweiz

    K.M. Rösler & O. Scheidegger

Authors
  1. K.M. Rösler
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. O. Scheidegger
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to K.M. Rösler.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Rösler, K., Scheidegger, O. Myositiden. Z Rheumatol 74, 496–506 (2015). https://doi.org/10.1007/s00393-014-1547-0

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-014-1547-0

Schlüsselwörter

Keywords

Search

Navigation