Main Article Content

Abstract

Dermatomyositis is an idiopathic inflammatory myopathy characterized by the presence of rash and moderate-to-severe muscle weakness secondary to inflammation of the muscle. It can be a difficult condition to treat. Systemic corticosteroids are the first choice of treatment. However, about a quarter of patients either fail to respond to steroids or develop steroid-related toxicity. Second-line agents such as azathioprine and methotrexate are then added either alone, or in combination with corticosteroids. Failure of the disease to respond to
second-line agents can then be a problem and this is often referred to as “refractory dermatomyositisâ€. Unfortunately, there is neither agreement nor wellestablished guidelines on the best regimen or combination of immunosuppressive agents in the case of refractory dermatomyositis.

Article Details

How to Cite
Setiyohadi, B., & Sinto, R. (2018). Experience with cyclophosphamide in the treatment of a young woman with refractory dermatomyositis. Indonesian Journal of Rheumatology, 3(1). https://doi.org/10.37275/ijr.v3i1.43

References

  1. Bernatsky S, Joseph L, Pineau CA, Bélisle P, Boivin JF, Banerjee D, et al. Estimating the prevalence of polymyositis and dermatomyositis from administrative data: age, sex and regional differences. Ann Rheum Dis. 2009; 68: 1192–6.
  2. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362: 71–82.
  3. Bendewald MJ, Wetter DA, Li X, Davis MDP. Incidence of dermatomyositis and clinically amyopathic dermatomyositis. Arch Dermatol. 2010; 146: 26–30.
  4. Wortmann RL. Idiopathic infl ammatory myopathies: clinical features. In: Klippel JH, Stone JH, Crofford LJ, White PH, editors. Primer on the rheumatic disease. 13th ed. New York: Springer; 2008. p. 363–7.
  5. Riley P, Maillard SM, Wedderburn LR, Woo P, Murray KJ, Pilkington CA. Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis:a review of effi cacy and safety. Rheumatology 2004: 43: 491–6.
  6. Ponyi A, Borgulya G, Constantin T, Vancsa A, Gergely L, Danko K. Functional outcome and quality of life in adult patients with idiopathic infl ammatory myositis. Rheumatology 2005; 44: 83–88.
  7. Oddis CV. Idiopathic infl ammatory myopathies: treatment and assessment. In: Klippel JH, Stone JH, Crofford LJ, White PH, editors. Primer on the rheumatic disease. 13th ed. New York: Springer; 2008. p.375–80.
  8. Cordeiro AC, Isenberg DA. Treatment of infl ammatory myopathies.Postgrad Med J. 2006; 82: 417–24.
  9. Lundberg IE, Vencovsky J, Dani L. Polymostitis, dermatomyositis, infl ammatory diseases of muscle and other myopathies. In: Bijlsma JWJ, Burmester GR, Silva JAP, Faarvang KL, Hachulla E, Mariette X. Eular compedium on rheumatic diseases. 1st ed. Kilchberg: BMJ Publishing Group; 2009. p. 297–313.
  10. McCann LJ, Garay SM, Ryan MM, Harris R, Riley P, Pilkington CA. Oropharyngeal dysphagia in juvenile dermatomyositis (JDM): an evaluation of videofl uoroscopy swallow study (VFSS) changes in relation to clinical symptoms and objective muscle scores. Rheumatology 2007; 46: 1363–6.
  11. Oh TH, Brumfi eld KA, Hoskin TL, Stolp KA, Murray JA, Basford JR. Dysphagia in infl ammatory myopathy: clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc. 2007; 82 : 441–7.
  12. Whallett AJ, Gillott TJ, Klocke R, Coppock JS. A case of refractory adult dermatomyositis. Br J Rheumatol 1998; 37: 544–5.
  13. Rider LG, Miller FW. Deciphering the clinical presentations, pathogenesis,
  14. and treatment of the idiopathic infl ammatory myopathies. JAMA 2011; 305: 183–90.
  15. Yamasaki Y, Yamada H, Yamasaki M, Ohkubo M, Azuma K, Matsuoka S. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/ dermatomyositis. rheumatology 2007; 46: 124–30.
  16. Choy EHS, Isenberg DA. Treatment of dermatomyositis and polymyositis. Rheumatology 2002; 41: 7–13.
  17. Katsifi s GE, Tzioufas AG. Ovarian failure in systemic lupus erythematosus patients treated with pulsed intravenous cyclophosphamide. Lupus 2004; 13: 673–8.
  18. Martin-Suarez I, Cruz DD, Mansoor M, Fernandes AP, Khamashta MA, Hughes GRV. Immunosuppressive treatment in severe connective tissue diseases: effects of low dose intravenous cyclophosphamide. Ann Rheum Dis. 1997; 56: 481–7.
  19. Martin F, Lauwerys B, Lefebvre C, Devogelaer JP, Houssiau. Side-effects of intravenous cyclophosphamide pulse therapy. Lupus 1997; 6: 254–7.
  20. Chen IJ, Tsai WP, Wu YJJ, Luo SF, Ho HH, Liou LB, et al. Infections in polymyositis and dermatomyositis: analysis of 192 cases. Rheumatology 2010; 49: 2429–37.
  21. Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B. Polymyositis and dermatomyositis: short-term and long-term outcome, and predictive factors of prognosis. J Rheumatol 2001; 28: 2230–7