Polymyositis and dermatomyositis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

Pharmacologic medical therapies for polymyositis and dermatomyositis include corticosteroids and disease-modifying antirheumatic drugs (DMARDs). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, glucocorticoids could be discontinued at 9 to 12 months. DMARDs might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of prednisone, adding or increasing dose of methotrexate or azathioprine. Rituximab, IVIg, and mycophenolate mofetil might be used in resistant diseases.

Medical Therapy

Polymyositis and dermatomyositis

  • 1 Initial disease
    • 1.1 Corticosteroids
      • Preferred regimen (1): Prednisone 1 mg/kg PO qd (maximum 80 mg daily) for 4-6 weeks and then prednisone should be tapered over 26 weeks to reach 5 mg/d. Prednisone should be tapered by 10 mg every week until 40 mg/day then tapered by 5 mg every week until 20 mg/d then tapered by 2.5 mg every week until 10 mg/day then tapered by 1 mg every two weeks until the patient reaches 5 mg/day.
      • Alternative regimen (1): Methylprednisolone 1000 mg IV qd for 3 days

Note (1): The side effects of corticosteroids include weight gain, redistribution of body fat, thinning of the skinosteoporosis, cataracts, and muscle weakness

  • Preferred regimen (1): Azathioprine 50 mg PO qd for 2 weeks and then increase the daily dose by 50 mg each week to 1.5 mg/kg/day (maximum 2.5 mg/kg/day)
  • Preferred regimen (2): Methotrexate 15 mg PO every week then increase the daily dose by 2.5 mg increments to 25 mg/week (side effects include hepatotoxicity and pulmonary toxicity.) 
  • Alternative regimen (1): Hydroxychloroquine 200-400 mg PO qd (for controlling skin disease)
  • 2 Recurrent disease
  • 3 Recurrent disease

References

  1. Khan, Sabiha; Christopher-Stine, Lisa (2011). "Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features". Rheumatic Disease Clinics of North America. 37 (2): 143–158. doi:10.1016/j.rdc.2011.01.001. ISSN 0889-857X.
  2. Dobloug, Cecilie; Garen, Torhild; Bitter, Helle; Stjärne, Johan; Stenseth, Guri; Grøvle, Lars; Sem, Marthe; Gran, Jan Tore; Molberg, Øyvind (2015). "Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort". Annals of the Rheumatic Diseases. 74 (8): 1551–1556. doi:10.1136/annrheumdis-2013-205127. ISSN 0003-4967.
  3. Chinoy, H.; Fertig, N.; Oddis, C. V; Ollier, W. E R; Cooper, R. G (2007). "The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis". Annals of the Rheumatic Diseases. 66 (10): 1345–1349. doi:10.1136/ard.2006.068502. ISSN 0003-4967.
  4. Dalakas, Marinos C; Hohlfeld, Reinhard (2003). "Polymyositis and dermatomyositis". The Lancet. 362 (9388): 971–982. doi:10.1016/S0140-6736(03)14368-1. ISSN 0140-6736.
  5. Douglas, William W.; Tazelaar, Henry D.; Hartman, Thomas E.; Hartman, Robert P.; Decker, Paul A.; Schroeder, Darrell R.; Ryu, Jay H. (2001). "Polymyositis–Dermatomyositis-associated Interstitial Lung Disease". American Journal of Respiratory and Critical Care Medicine. 164 (7): 1182–1185. doi:10.1164/ajrccm.164.7.2103110. ISSN 1073-449X.