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Opinion statement

Patients with polymyositis or dermatomyositis should be treated with prednisone (approximately 1 mg/kg/d) for an initial period of 4 to 6 weeks. Once improvement occurs, the dose should be tapered and converted to an alternate-day regimen, which should be continued for at least 12 months. Methotrexate or azathioprine should be administered concomitantly to patients in whom there is inadequate control. The early introduction of one of these drugs allows more rapid reduction in the dose of prednisone and helps to avert serious side effects. Intravenous immunoglobulin therapy is indicated for patients who have immunodeficiency, who are unable to tolerate immunosuppressive drugs, whose conditions are deteriorating, or who have severe relapses. Cyclosporine or cyclophosphamide may be effective for resistant disease. Patients with inclusion body myositis should undergo a 3- to 6-month trial of prednisone, alone or in combination with methotrexate or azathioprine. Maintenance doses of these drugs should be continued if the patient’s condition improves or stabilizes.

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References and Recommended Reading

  1. Walton JN, Adams PD: Polymyositis. London: Churchill Livingstone; 1958.

    Google Scholar 

  2. Whitaker JN, Engel WK: Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy. N Engl J Med 1972, 286:333–338.

    Article  PubMed  CAS  Google Scholar 

  3. Kissel JT, Mendell JR, Rammohan KW: Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986, 314:329–334.

    Article  PubMed  CAS  Google Scholar 

  4. Emslie-Smith AM, Engel AG: Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol 1990, 27:343–356.

    Article  PubMed  CAS  Google Scholar 

  5. Engel AG, Arahata K: Mononuclear cells in myopathies. Hum Pathol 1986, 17:704–721.

    Article  PubMed  CAS  Google Scholar 

  6. Yunis EJ, Samaha FJ: Inclusion body myositis. Lab Invest 1971, 25:240–248.

    PubMed  CAS  Google Scholar 

  7. Adams RD, Samaha FJ, Kakulas BA: A myopathy with cellular inclusions. Trans Am Neurol Assoc 1965, 90:213–216.

    PubMed  CAS  Google Scholar 

  8. Dalakas MC: Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med 1991, 325:1487–1498.

    Article  PubMed  CAS  Google Scholar 

  9. Mendell JR: Sporadic inclusion body myositis: clinical and laboratory features and diagnostic criteria. In Inclusion Body Myositis and Myopathies. Edited by Askanas V, Serratrice G, Engel WK. Cambridge, UK: Cambridge University Press; 1998.

    Google Scholar 

  10. Oldfors A, Moslemi A-R, Holme E, et al.: Mitochondrial alterations in sporadic inclusion body myositis. In Inclusion Body Myositis and Myopathies. Edited by Askanas V, Serratrice G, Engel WK. Cambridge, UK: Cambridge University Press; 1998.

    Google Scholar 

  11. Garlepp MJ, Mastaglia FL: Inclusion body myositis. J Neurol Neurosurg Psychiatry 1996, 60:251–255.

    PubMed  CAS  Google Scholar 

  12. Mastaglia FL, Phillips BA, Zilko PJ: Treatment of inflammatory myopathy. Muscle Nerve 1997, 20:651–664. A review of the treatment of inflammatory myopathies.

    Article  PubMed  CAS  Google Scholar 

  13. Kremer JM: Is methotrexate oncogenic in patients with rheumatoid arthritis? Semin Arthritis Rheumatol 1997, 26:785–787.

    Article  CAS  Google Scholar 

  14. 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.

    Article  PubMed  CAS  Google Scholar 

  15. Mastaglia FL, Phillips BA, Zilko PJ: Immunoglobulin therapy in inflammatory myopathies. J Neurol Neurosurg Psychiatry 1998, 65:107–110. A recent trial of intravenous immunoglobulin in various forms of inflammatory myopathy.

    PubMed  CAS  Google Scholar 

  16. Cherin P, Herson S, Wechsler B, et al.: Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients. Am J Med 1991, 91:162–168.

    Article  PubMed  CAS  Google Scholar 

  17. Cherin P, Piette JC, Wechsler B, et al.: Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients. J Rheumatol 1994, 21:1092–1097.

    PubMed  CAS  Google Scholar 

  18. Dalakas MC: Retroviruses and inflammatory myopathies in humans and primates. Bailliere’s Clin Neurol 1993, 2:659–691.

    CAS  Google Scholar 

  19. Dau PC: Plasmapheresis in idiopathic inflammatory myopathy. Arch Neurol 1981, 38:544–552.

    PubMed  CAS  Google Scholar 

  20. Miller FW, Leitman SF, Cronin ME, et al.: Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992, 326:1380–1384.

    Article  PubMed  CAS  Google Scholar 

  21. Heckmatt J, Saunders C, Peters AM, et al.: Cyclosporine in juvenile dermatomyositis. Lancet 1989, 1:1063–1066.

    Article  PubMed  CAS  Google Scholar 

  22. Engel WK, Lighter AS, Galdi AP: Polymyositis: remarkable response to total body irradiation. Lancet 1981, 1:658.

    PubMed  CAS  Google Scholar 

  23. Lane RJM, Hudgson P: Thymectomy in polymyositis [letter]. Lancet 1984, 1:626–627.

    Article  Google Scholar 

  24. Rosenberg NL, Ringel SP: Adult polymyositis and dermatomyositis. In Inflammatory Diseases of Muscle. Edited by Mastaglia FL. Oxford: Blackwell Scientific Publications; 1988.

    Google Scholar 

  25. Sayers ME, Chou SM, Calabrese LH: Inclusion body myositis: analysis of 32 cases. J Rheumatol 1992, 19:1385–1389.

    PubMed  CAS  Google Scholar 

  26. Leff RL, Miller FW, Hicks J, et al.: The treatment of inclusion body myositis: a retrospective review and a randomized, prospective trial of immunosuppressive therapy. Medicine 1993, 72:225–235.

    Article  PubMed  CAS  Google Scholar 

  27. Dalakas MC, Sonies B, Dambrosia J, et al.: Treatment of inclusion body myositis with IVIg: a double-blind placebo-controlled study. Neurology 1997, 48:712–716. This is the only placebo-controlled trial of intravenous immunoglobulin in inclusion body myositis.

    PubMed  CAS  Google Scholar 

  28. Soueidan SA, Dalakas MC: Treatment of inclusion body myositis with high-dose intravenous immunoglobulin. Neurology 1993, 43:876–879.

    PubMed  CAS  Google Scholar 

  29. Amato AA, Barohn RJ, Jackson CE, et al.: Inclusion body myositis: treatment with intravenous immunoglobulin. Neurology 1994, 4:1516–1518.

    Google Scholar 

  30. Matsubara S, Sawa Y, Takamori M, et al.: Pulsed intravenous methylprednisolone combined with oral steroids as the initial treatment of inflammatory myopathies [letter]. J Neurol Neurosurg Psychiatry 1994, 7:1008.

    Google Scholar 

  31. Mastaglia FL, Laing BA, Zilko P: Treatment of inflammatory myopathies. Bailliere’s Clin Neurol 1993, 2:717–740.

    CAS  Google Scholar 

  32. Adachi JD, Benson WG, Brown J, et al.: Intermittent etidronate therapy to prevent corticosteroid-induced osteoporosis. N Engl J Med 1997, 337:382–387.

    Article  PubMed  CAS  Google Scholar 

  33. Saag KG, Emkey R, Schnitzer TJ, et al.: Alendronate for the prevention and treatment of glucocorticoidinduced osteoporosis. N Engl J Med 1998, 339:292–299.

    Article  PubMed  CAS  Google Scholar 

  34. Joffe MM, Love LA, Leff RL, et al.: Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisolone, azathioprine and methotrexate and a comparison of their efficacy. Am J Med 1993, 94:379–387.

    Article  PubMed  CAS  Google Scholar 

  35. Kremer JM, Alarcon GS, Lightfoot RW Jr., et al.: Methotrexate for rheumatoid arthritis. Arthritis Rheumatol 1994, 37:316–325.

    Article  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  37. Confraveaux C, Saddier P, Grimaud J, et al.: Risk of cancer from azathioprine therapy in multiple sclerosis: a case-control study. Neurology 1996, 46:1607–1612.

    Google Scholar 

  38. Black AJ, McLeod HL, Capell HA, et al.: Thiopurine methyltransferase genotype predicts therapy-limiting severe toxicity from azathioprine. Ann Intern Med 1998, 29:716–718.

    Google Scholar 

  39. Haga HJ, D’Cruz D, Asherson R, Hughes GR: Short term effects of intravenous pulses of cyclophosphamide in the treatment of connective tissue disease crises. Ann Rheumatol Dis 1992, 51:885–888.

    Article  CAS  Google Scholar 

  40. Freedman A, Ehrlich RM, Ljung BM: Prevention of cyclophosphamide cystitis with 2-mercaptoethane sodium sulfonate: a histologic study. J Urol 1984, 132:580–582.

    PubMed  CAS  Google Scholar 

  41. Mansur AY, Topaloglu H, Jungbluth H, et al.: Cyclosporin treatment in juvenile dermatomyositis: a review of 26 cases. Muscle Nerve 1998, 7(suppl):S150.

    Google Scholar 

  42. Leuck CJ, Trend PT, Swash M: Cyclosporin in the management of polymyositis and dermatomyositis. J Neurol Neurosurg Psychiatry 1991, 54:1007–1008.

    Article  Google Scholar 

  43. Grau JM, Herrero C, Casademont J, et al.: Cyclosporin A as first choice for dermatomyositis. J Rheumatol 1994, 21:381–382.

    PubMed  CAS  Google Scholar 

  44. Bertorini TE, Nance AM, Horner LH, et al.: Complications of intravenous gammaglobulin in neuromuscular and other diseases. Muscle Nerve 1996, 19:388–391.

    Article  PubMed  CAS  Google Scholar 

  45. Hicks JE: Role of rehabilitation in the management of myopathies. Curr Opin Rheumatol 1998, 10:548–555.

    Article  PubMed  CAS  Google Scholar 

  46. Escalante A, Miller L, Bedardmore TD: Resistive exercise in the rehabilitation of polymyositis/dermatomyositis. J Rheumatol 1993, 20:1340–1344.

    PubMed  CAS  Google Scholar 

  47. Horber FF, Schiedegger JR, Grunig BE, Frey FJ: Thigh muscle mass and function in patients treated with glucocorticoids. Eur J Clin Invest 1985, 15:302–307.

    PubMed  CAS  Google Scholar 

  48. Wiesinger GR, Quittan M, Aringer M, et al.: Improvement of physical fitness and muscle strength in polymyositis/dermatomyositis patients by a training programme. Br J Rheumatol 1998, 37:196–200.

    Article  PubMed  CAS  Google Scholar 

  49. Spector SA, Lemmer JT, Koffman BM, et al.: Safety and efficacy of strength training in patients with sporadic inclusion body myositis. Muscle Nerve 1997, 20:1242–1248.

    Article  PubMed  CAS  Google Scholar 

  50. Zantos D, Zhang Y, Felson D: The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol 1994, 21:1855–1859.

    PubMed  CAS  Google Scholar 

  51. Danon MJ, Friedman M: Inclusion body myositis associated with progressive dysphagia: treatment with cricopharyngeal myotomy. Can J Neurol Sci 1989, 16:436–438.

    PubMed  CAS  Google Scholar 

  52. Tyndall A: Hematopoietic stem cell transplantation in rheumatic diseases other than systemic sclerosis and systemic lupus erythematosus. J Rheumatol 1997, 48:94–97.

    CAS  Google Scholar 

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Mastaglia, F.L., Phillips, B.A. & Zilko, P.J. Inflammatory myopathy. Curr Treat Options Neurol 1, 263–272 (1999). https://doi.org/10.1007/s11940-999-0008-6

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