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Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis

Clare Hutchinson, MDCM, FRCPC
Brian M Feldman, MD, MSc, FRCPC
Section Editor
Marc C Patterson, MD, FRACP
Deputy Editor
Elizabeth TePas, MD, MS


Juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) are autoimmune myopathies of childhood. JDM is primarily a capillary vasculopathy, whereas JPM involves direct T cell invasion of muscle fibers similar to that seen in adult polymyositis [1-3]. However, as the diagnostic tools become more sophisticated (eg, biopsies that demonstrate inclusion body myositis or inflammatory dystrophies, or autoantibodies that are markers of particular types of myositis such as anti-signal recognition particle [SRP] and necrotizing myopathy), fewer patients are diagnosed with JPM, calling into question whether JPM is a specific entity. (See "Clinical manifestations of dermatomyositis and polymyositis in adults".)

The treatment, complications, natural history, and prognosis of JDM and JPM are reviewed here. The pathogenesis, clinical manifestations, and diagnosis of these disorders are discussed elsewhere. (See "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Juvenile dermatomyositis and polymyositis: Diagnosis".)


There are few data from randomized, controlled trials to guide management decisions in the children with JDM or JPM. Until better data exist, we use the approach discussed below, based upon observational studies and our clinical experience. The goals of treatment include control of the underlying inflammatory myositis and prevention and/or treatment of complications (eg, contractures and calcinosis).

Initial treatment — The intensity of initial therapy increases with increasing severity of the symptoms [4], which range from mild disease with muscle weakness and cutaneous manifestations to serious, life-threatening weakness, internal organ damage, and ulcerative skin lesions. In a 2008 survey of North American pediatric rheumatologists, the majority of respondents used a combination of glucocorticoids and methotrexate to treat children with JDM [5]. However, the dose, route of administration, and the use of adjunctive therapy (eg, intravenous immunoglobulin [IVIG] and hydroxychloroquine) varied. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) has proposed several treatment strategies for initial therapy for JDM based upon a North American survey of practice [6]. Our approach is consistent with the CARRA treatment strategies.

Mild to moderate disease — The initial treatment used for the majority of children with JDM or JPM is a combination of high-dose oral prednisone (2 mg/kg per day, maximum 80 mg/day, in divided doses given twice daily) and methotrexate (15 mg/m2, maximum 25 mg/dose once weekly, administered as a subcutaneous injection). Children who receive methotrexate should also be given folic acid at 1 mg per day to limit methotrexate toxicity.

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Literature review current through: Dec 2017. | This topic last updated: May 17, 2017.
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  1. Stringer E, Feldman BM. Advances in the treatment of juvenile dermatomyositis. Curr Opin Rheumatol 2006; 18:503.
  2. Miles L, Bove KE, Lovell D, et al. Predictability of the clinical course of juvenile dermatomyositis based on initial muscle biopsy: a retrospective study of 72 patients. Arthritis Rheum 2007; 57:1183.
  3. Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008; 371:2201.
  4. Stringer E, Bohnsack J, Bowyer SL, et al. Treatment approaches to juvenile dermatomyositis (JDM) across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey. J Rheumatol 2010; 37:1953.
  5. Stringer E, Ota S, Bohnsack J, et al. Diversity of treatment approaches to juvenile dermatomyositis (JDM) across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM treatment study. Arthritis Rheum 2008; 58:S255.
  6. Huber AM, Giannini EH, Bowyer SL, et al. Protocols for the initial treatment of moderately severe juvenile dermatomyositis: results of a Children's Arthritis and Rheumatology Research Alliance Consensus Conference. Arthritis Care Res (Hoboken) 2010; 62:219.
  7. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Invest Dermatol 1993; 100:124S.
  8. Plamondon S, Dent PB. Juvenile amyopathic dermatomyositis: results of a case finding descriptive survey. J Rheumatol 2000; 27:2031.
  9. Stonecipher MR, Jorizzo JL, White WL, et al. Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis? J Am Acad Dermatol 1993; 28:951.
  10. Bowyer SL, Blane CE, Sullivan DB, Cassidy JT. Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr 1983; 103:882.
  11. Huber A, Feldman BM. Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going? Curr Rheumatol Rep 2005; 7:441.
  12. Sullivan DB, Cassidy JT, Petty RE, Burt A. Prognosis in childhood dermatomyositis. J Pediatr 1972; 80:555.
  13. Spencer CH, Hanson V, Singsen BH, et al. Course of treated juvenile dermatomyositis. J Pediatr 1984; 105:399.
  14. Tabarki B, Ponsot G, Prieur AM, Tardieu M. Childhood dermatomyositis: clinical course of 36 patients treated with low doses of corticosteroids. Eur J Paediatr Neurol 1998; 2:205.
  15. Rouster-Stevens KA, Gursahaney A, Ngai KL, et al. Pharmacokinetic study of oral prednisolone compared with intravenous methylprednisolone in patients with juvenile dermatomyositis. Arthritis Rheum 2008; 59:222.
  16. Lang B, Dooley J. Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis. J Pediatr 1996; 128:429.
  17. Seshadri R, Feldman BM, Ilowite N, et al. The role of aggressive corticosteroid therapy in patients with juvenile dermatomyositis: a propensity score analysis. Arthritis Rheum 2008; 59:989.
  18. Klein-Gitelman MS, Waters T, Pachman LM. The economic impact of intermittent high-dose intravenous versus oral corticosteroid treatment of juvenile dermatomyositis. Arthritis Care Res 2000; 13:360.
  19. Ramanan AV, Campbell-Webster N, Ota S, et al. The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum 2005; 52:3570.
  20. Al-Mayouf S, Al-Mazyed A, Bahabri S. Efficacy of early treatment of severe juvenile dermatomyositis with intravenous methylprednisolone and methotrexate. Clin Rheumatol 2000; 19:138.
  21. Ruperto N, Pistorio A, Oliveira S, et al. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial. Lancet 2016; 387:671.
  22. Fisler RE, Liang MG, Fuhlbrigge RC, et al. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 2002; 47:505.
  23. Levy DM, Bingham CA, Kahn PJ, et al. Favorable outcome of juvenile dermatomyositis treated without systemic corticosteroids. J Pediatr 2010; 156:302.
  24. Riley P, Maillard SM, Wedderburn LR, et al. Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety. Rheumatology (Oxford) 2004; 43:491.
  25. Smith RL, Sundberg J, Shamiyah E, et al. Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. J Rheumatol 2004; 31:1644.
  26. Schmeling H, Stephens S, Goia C, et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford) 2011; 50:885.
  27. Guzmán J, Petty RE, Malleson PN. Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes. J Rheumatol 1994; 21:739.
  28. Rider LG, Aggarwal R, Pistorio A, et al. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol 2017; 69:911.
  29. CHQ: Child Heath Questionnaire. https://www.healthactchq.com/chq.php.
  30. Bode RK, Klein-Gitelman MS, Miller ML, et al. Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence. Arthritis Rheum 2003; 49:7.
  31. Sansome A, Dubowitz V. Intravenous immunoglobulin in juvenile dermatomyositis--four year review of nine cases. Arch Dis Child 1995; 72:25.
  32. Collet E, Dalac S, Maerens B, et al. Juvenile dermatomyositis: treatment with intravenous gammaglobulin. Br J Dermatol 1994; 130:231.
  33. Al-Mayouf SM, Laxer RM, Schneider R, et al. Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety. J Rheumatol 2000; 27:2498.
  34. Lang BA, Laxer RM, Murphy G, et al. Treatment of dermatomyositis with intravenous gammaglobulin. Am J Med 1991; 91:169.
  35. Lam CG, Manlhiot C, Pullenayegum EM, Feldman BM. Efficacy of intravenous Ig therapy in juvenile dermatomyositis. Ann Rheum Dis 2011; 70:2089.
  36. Roifman CM, Schaffer FM, Wachsmuth SE, et al. Reversal of chronic polymyositis following intravenous immune serum globulin therapy. JAMA 1987; 258:513.
  37. Yang MC, Lee JH, Yang YH, Chiang BL. Improvement of juvenile dermatomyositis with calcinosis universalis after treatment with intravenous immunoglobulin. Int J Rheum Dis 2008; 11:77.
  38. Manlhiot C, Tyrrell PN, Liang L, et al. Safety of intravenous immunoglobulin in the treatment of juvenile dermatomyositis: adverse reactions are associated with immunoglobulin A content. Pediatrics 2008; 121:e626.
  39. Heckmatt J, Hasson N, Saunders C, et al. Cyclosporin in juvenile dermatomyositis. Lancet 1989; 1:1063.
  40. Zeller V, Cohen P, Prieur AM, Guillevin L. Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases. J Rheumatol 1996; 23:1424.
  41. Reiff A, Rawlings DJ, Shaham B, et al. Preliminary evidence for cyclosporin A as an alternative in the treatment of recalcitrant juvenile rheumatoid arthritis and juvenile dermatomyositis. J Rheumatol 1997; 24:2436.
  42. Kobayashi I, Yamada M, Takahashi Y, et al. Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A. Rheumatology (Oxford) 2003; 42:371.
  43. Dantzig P. Juvenile dermatomyositis treated with cyclosporine. J Am Acad Dermatol 1990; 22:310.
  44. Cooper MA, Willingham DL, Brown DE, et al. Rituximab for the treatment of juvenile dermatomyositis: a report of four pediatric patients. Arthritis Rheum 2007; 56:3107.
  45. Bader-Meunier B, Decaluwe H, Barnerias C, et al. Safety and efficacy of rituximab in severe juvenile dermatomyositis: results from 9 patients from the French Autoimmunity and Rituximab registry. J Rheumatol 2011; 38:1436.
  46. Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 2013; 65:314.
  47. Riley P, McCann LJ, Maillard SM, et al. Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis. Rheumatology (Oxford) 2008; 47:877.
  48. Arabshahi B, Silverman RA, Jones OY, Rider LG. Abatacept and sodium thiosulfate for treatment of recalcitrant juvenile dermatomyositis complicated by ulceration and calcinosis. J Pediatr 2012; 160:520.
  49. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol 2006; 142:65.
  50. Rouster-Stevens KA, Morgan GA, Wang D, Pachman LM. Mycophenolate mofetil: a possible therapeutic agent for children with juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62:1446.
  51. Yamada A, Ohshima Y, Omata N, et al. Steroid-sparing effect of tacrolimus in a patient with juvenile dermatomyositis presenting poor bioavailability of cyclosporine A. Eur J Pediatr 2004; 163:561.
  52. Hassan J, van der Net JJ, van Royen-Kerkhof A. Treatment of refractory juvenile dermatomyositis with tacrolimus. Clin Rheumatol 2008; 27:1469.
  53. Hollar CB, Jorizzo JL. Topical tacrolimus 0.1% ointment for refractory skin disease in dermatomyositis: a pilot study. J Dermatolog Treat 2004; 15:35.
  54. García-Doval I, Cruces M. Topical tacrolimus in cutaneous lesions of dermatomyositis: lack of effect in side-by-side comparison in five patients. Dermatology 2004; 209:247.
  55. Olson NY, Lindsley CB. Adjunctive use of hydroxychloroquine in childhood dermatomyositis. J Rheumatol 1989; 16:1545.
  56. Klepper SE. Exercise in pediatric rheumatic diseases. Curr Opin Rheumatol 2008; 20:619.
  57. Hicks JE, Drinkard B, Summers RM, Rider LG. Decreased aerobic capacity in children with juvenile dermatomyositis. Arthritis Rheum 2002; 47:118.
  58. Habers GE, Bos GJ, van Royen-Kerkhof A, et al. Muscles in motion: a randomized controlled trial on the feasibility, safety and efficacy of an exercise training programme in children and adolescents with juvenile dermatomyositis. Rheumatology (Oxford) 2016; 55:1251.
  59. Maillard SM, Jones R, Owens CM, et al. Quantitative assessments of the effects of a single exercise session on muscles in juvenile dermatomyositis. Arthritis Rheum 2005; 53:558.
  60. Huber AM, Lang B, LeBlanc CM, et al. Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 2000; 43:541.
  61. Deakin CT, Yasin SA, Simou S, et al. Muscle Biopsy Findings in Combination With Myositis-Specific Autoantibodies Aid Prediction of Outcomes in Juvenile Dermatomyositis. Arthritis Rheumatol 2016; 68:2806.
  62. Ravelli A, Trail L, Ferrari C, et al. Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res (Hoboken) 2010; 62:63.
  63. Sanner H, Sjaastad I, Flatø B. Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool. Rheumatology (Oxford) 2014; 53:1578.
  64. Santiago RA, Silva CA, Caparbo VF, et al. Bone mineral apparent density in juvenile dermatomyositis: the role of lean body mass and glucocorticoid use. Scand J Rheumatol 2008; 37:40.
  65. Huber AM, Gaboury I, Cabral DA, et al. Prevalent vertebral fractures among children initiating glucocorticoid therapy for the treatment of rheumatic disorders. Arthritis Care Res (Hoboken) 2010; 62:516.
  66. Rouster-Stevens KA, Langman CB, Price HE, et al. RANKL:osteoprotegerin ratio and bone mineral density in children with untreated juvenile dermatomyositis. Arthritis Rheum 2007; 56:977.
  67. Alsufyani KA, Ortiz-Alvarez O, Cabral DA, et al. Bone mineral density in children and adolescents with systemic lupus erythematosus, juvenile dermatomyositis, and systemic vasculitis: relationship to disease duration, cumulative corticosteroid dose, calcium intake, and exercise. J Rheumatol 2005; 32:729.
  68. Warady BD, Lindsley CB, Robinson FG, Lukert BP. Effects of nutritional supplementation on bone mineral status of children with rheumatic diseases receiving corticosteroid therapy. J Rheumatol 1994; 21:530.
  69. Bianchi ML, Cimaz R, Bardare M, et al. Efficacy and safety of alendronate for the treatment of osteoporosis in diffuse connective tissue diseases in children: a prospective multicenter study. Arthritis Rheum 2000; 43:1960.
  70. Pachman LM, Hayford JR, Chung A, et al. Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. J Rheumatol 1998; 25:1198.
  71. McCann LJ, Juggins AD, Maillard SM, et al. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford) 2006; 45:1255.
  72. Sallum AM, Pivato FC, Doria-Filho U, et al. Risk factors associated with calcinosis of juvenile dermatomyositis. J Pediatr (Rio J) 2008; 84:68.
  73. Blane CE, White SJ, Braunstein EM, et al. Patterns of calcification in childhood dermatomyositis. AJR Am J Roentgenol 1984; 142:397.
  74. Pachman LM, Liotta-Davis MR, Hong DK, et al. TNFalpha-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor alpha, disease duration, and pathologic calcifications. Arthritis Rheum 2000; 43:2368.
  75. Tansley SL, Betteridge ZE, Shaddick G, et al. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology (Oxford) 2014; 53:2204.
  76. Marhaug G, Shah V, Shroff R, et al. Age-dependent inhibition of ectopic calcification: a possible role for fetuin-A and osteopontin in patients with juvenile dermatomyositis with calcinosis. Rheumatology (Oxford) 2008; 47:1031.
  77. Eddy MC, Leelawattana R, McAlister WH, Whyte MP. Calcinosis universalis complicating juvenile dermatomyositis: resolution during probenecid therapy. J Clin Endocrinol Metab 1997; 82:3536.
  78. Harel L, Harel G, Korenreich L, et al. Treatment of calcinosis in juvenile dermatomyositis with probenecid: the role of phosphorus metabolism in the development of calcifications. J Rheumatol 2001; 28:1129.
  79. Nakamura H, Kawakami A, Ida H, et al. Efficacy of probenecid for a patient with juvenile dermatomyositis complicated with calcinosis. J Rheumatol 2006; 33:1691.
  80. Ichiki Y, Akiyama T, Shimozawa N, et al. An extremely severe case of cutaneous calcinosis with juvenile dermatomyositis, and successful treatment with diltiazem. Br J Dermatol 2001; 144:894.
  81. Oliveri MB, Palermo R, Mautalen C, Hübscher O. Regression of calcinosis during diltiazem treatment in juvenile dermatomyositis. J Rheumatol 1996; 23:2152.
  82. Wananukul S, Pongprasit P, Wattanakrai P. Calcinosis cutis presenting years before other clinical manifestations of juvenile dermatomyositis: report of two cases. Australas J Dermatol 1997; 38:202.
  83. Wang WJ, Lo WL, Wong CK. Calcinosis cutis in juvenile dermatomyositis: remarkable response to aluminum hydroxide therapy. Arch Dermatol 1988; 124:1721.
  84. Ambler GR, Chaitow J, Rogers M, et al. Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy. J Rheumatol 2005; 32:1837.
  85. Marco Puche A, Calvo Penades I, Lopez Montesinos B. Effectiveness of the treatment with intravenous pamidronate in calcinosis in juvenile dermatomyositis. Clin Exp Rheumatol 2010; 28:135.
  86. Al-Mayouf SM, Alsonbul A, Alismail K. Localized calcinosis in juvenile dermatomyositis: successful treatment with intralesional corticosteroids injection. Int J Rheum Dis 2010; 13:e26.
  87. Wu JJ, Metz BJ. Calcinosis cutis of juvenile dermatomyositis treated with incision and drainage. Dermatol Surg 2008; 34:575.
  88. Downey EC Jr, Woolley MM, Hanson V. Required surgical therapy in the pediatric patient with dermatomyositis. Arch Surg 1988; 123:1117.
  89. Stockton D, Doherty VR, Brewster DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer 2001; 85:41.
  90. Christen-Zaech S, Seshadri R, Sundberg J, et al. Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis. Arthritis Rheum 2008; 58:571.
  91. BITNUM S, DAESCHNER CW Jr, TRAVIS LB, et al. DERMATOMYOSITIS. J Pediatr 1964; 64:101.
  92. Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum 1982; 25:126.
  93. Sanner H, Gran JT, Sjaastad I, Flatø B. Cumulative organ damage and prognostic factors in juvenile dermatomyositis: a cross-sectional study median 16.8 years after symptom onset. Rheumatology (Oxford) 2009; 48:1541.