J Rheum Dis.  2022 Jan;29(1):14-21. 10.4078/jrd.2022.29.1.14.

Juvenile Dermatomyositis

Affiliations
  • 1Department of Pediatrics, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Juvenile dermatomyositis (JDM) is a systemic capillary vasculopathy. Patients present with proximal muscle weakness, raised muscle enzymes, and pathognomic skin rashes such as heliotrope rash, Gottron’s papules. Main complications are calcinosis, lipodystrophy, osteoporosis. Complement-mediated damage of vessels is a major mechanism. Magnetic resonance imaging is currently widely used to diagnosis of JDM. The goals of treatment are to control inflammatory myositis and prevent disease complication. Early, aggressive treatment of JDM associated with a better prognosis. High-dose corticosteroids in combination with methotrexate is the mainstay of treatment. The course of JDM is variable.

Keyword

Juvenile dermatomyositis; Muscle weakness; Rash

Figure

  • Figure 1 Classification tree for subgroups of idiopathic inflammatory myopathies (IIM). PM: polymyositis, IBM: inclusion body myositis, ADM: amyopathic dermatomyositis, DM: dermatomyositis, JDM: juvenile dermatomyositis, EULAR/ACR: European League Against Rheumatism/American College of Rheumatology. *The PM subset includes immune-mediated necrotizing myopathies (IMNM).


Reference

1. Mathiesen PR, Zak M, Herlin T, Nielsen SM. 2010; Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients. Clin Exp Rheumatol. 28:782–9.
2. Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H, et al. 2014; Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry. Arthritis Care Res (Hoboken). 66:404–10. DOI: 10.1002/acr.22142. PMID: 23983017. PMCID: PMC4078654.
Article
3. Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A, et al. 2003; US incidence of juvenile dermatomyositis, 1995-1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry. Arthritis Rheum. 49:300–5. DOI: 10.1002/art.11122. PMID: 12794783.
Article
4. Symmons DP, Sills JA, Davis SM. 1995; The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 34:732–6. DOI: 10.1093/rheumatology/34.8.732. PMID: 7551657.
Article
5. Mamyrova G, O'Hanlon TP, Monroe JB, Carrick DM, Malley JD, Adams S, et al. 2006; Immunogenetic risk and protective factors for juvenile dermatomyositis in Caucasians. Arthritis Rheum. 54:3979–87. DOI: 10.1002/art.22216. PMID: 17133612. PMCID: PMC2063456.
Article
6. Khanna S, Reed AM. 2010; Immunopathogenesis of juvenile dermatomyositis. Muscle Nerve. 41:581–92. DOI: 10.1002/mus.21669. PMID: 20405498.
Article
7. Tse S, Lubelsky S, Gordon M, Al Mayouf SM, Babyn PS, Laxer RM, et al. 2001; The arthritis of inflammatory childhood myositis syndromes. J Rheumatol. 28:192–7.
8. Barth Z, Witczak BN, Flatø B, Koller A, Sjaastad I, Sanner H. 2018; Assessment of microvascular abnormalities by nailfold capillaroscopy in juvenile dermatomyositis after medium- to long-term followup. Arthritis Care Res (Hoboken). 70:768–76. DOI: 10.1002/acr.23338. PMID: 28805016.
Article
9. Huber AM, Lang B, LeBlanc CM, Birdi N, Bolaria RK, Malleson P, et al. 2000; Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum. 43:541–9. DOI: 10.1002/1529-0131(200003)43:3<541::AID-ANR9>3.0.CO;2-T.
Article
10. Bohan A, Peter JB. 1975; Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 292:344–7. DOI: 10.1056/NEJM197502132920706. PMID: 1090839.
11. Brown VE, Pilkington CA, Feldman BM, Davidson JE. Network for Juvenile Dermatomyositis, Paediatric Rheumatology European Society (PReS). 2006; An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford). 45:990–3. DOI: 10.1093/rheumatology/kel025. PMID: 16467366.
Article
12. Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. 2017; 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Arthritis Rheumatol. 69:2271–82. DOI: 10.1002/art.40320. PMID: 29106061. PMCID: PMC5846474.
13. Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD. 2013; Clinical features and disease course of patients with juvenile dermatomyositis. Int J Rheum Dis. 16:561–7. DOI: 10.1111/1756-185X.12107. PMID: 24164844.
Article
14. Rider LG, Shah M, Mamyrova G, Huber AM, Rice MM, Targoff IN, et al. 2013; The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore). 92:223–43. DOI: 10.1097/MD.0b013e31829d08f9. PMID: 23877355. PMCID: PMC3721421.
Article
15. Bowyer SL, Blane CE, Sullivan DB, Cassidy JT. 1983; Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr. 103:882–8. DOI: 10.1016/S0022-3476(83)80706-9.
Article
16. Ramanan AV, Campbell-Webster N, Ota S, Parker S, Tran D, Tyrrell PN, et al. 2005; The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum. 52:3570–8. DOI: 10.1002/art.21378. PMID: 16255046.
Article
17. Ruperto N, Pistorio A, Oliveira S, Zulian F, Cuttica R, Ravelli A, et al. 2016; Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial. Lancet. 387:671–8. DOI: 10.1016/S0140-6736(15)01021-1.
Article
18. Deakin CT, Campanilho-Marques R, Simou S, Moraitis E, Wedderburn LR, Pullenayegum E, et al. 2018; Efficacy and safety of cyclophosphamide treatment in severe juvenile dermatomyositis shown by marginal structural modeling. Arthritis Rheumatol. 70:785–93. DOI: 10.1002/art.40418. PMID: 29342499. PMCID: PMC5947636.
Article
19. Rouster-Stevens KA, Morgan GA, Wang D, Pachman LM. 2010; Mycophenolate mofetil: a possible therapeutic agent for children with juvenile dermatomyositis. Arthritis Care Res (Hoboken). 62:1446–51. DOI: 10.1002/acr.20269. PMID: 20521307. PMCID: PMC2952049.
Article
20. Cooper MA, Willingham DL, Brown DE, French AR, Shih FF, White AJ. 2007; Rituximab for the treatment of juvenile dermatomyositis: a report of four pediatric patients. Arthritis Rheum. 56:3107–11. DOI: 10.1002/art.22856. PMID: 17763414.
Article
21. Maillard SM, Jones R, Owens CM, Pilkington C, Woo PM, Wedderburn LR, et al. 2005; Quantitative assessments of the effects of a single exercise session on muscles in juvenile dermatomyositis. Arthritis Rheum. 53:558–64. DOI: 10.1002/art.21332. PMID: 16082634.
Article
22. Orandi AB, Baszis KW, Dharnidharka VR, Huber AM, Hoeltzel MF. CARRA Juvenile Myositis subgroup. 2017; Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). Pediatr Rheumatol Online J. 15:71. DOI: 10.1186/s12969-017-0199-4. PMID: 28934971. PMCID: PMC5609060.
Article
23. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, et al. 2004; Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 50:1595–603. DOI: 10.1002/art.20179. PMID: 15146430.
Article
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