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Juvenile Dermatomyositis

  • Ann M. Reed
  • Clarissa A. Pilkington
  • Brian M. Feldman
  • Lauren M. Pachman
  • Lisa G. Rider

Abstract

Children with inflammatory myopathies have clinical and laboratory features, as well as risk factors and outcomes that overlap with their adult counterparts. However, a number of important differences exist between pediatric and adult disease. Juvenile dermatomyositis (JDM), the most common of this group of illnesses in children, has an incidence of approximately 3.2 cases/million children/year. JDM has a gender ratio of 2 girls:1 boy. JDM has a mean age of 6.7 years at disease onset. The duration of active disease before the time of diagnosis alters the patients' features at presentation as well as their clinical outcomes. Environmental and genetic factors affect the child's susceptibility to these conditions and also alter the inflammatory response, adding heterogeneity to disease pathophysiology.

Keywords

Celiac Disease Juvenile Idiopathic Arthritis Interstitial Lung Disease Mixed Connective Tissue Disease Inflammatory Myopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Baechler EC, Bauer JW, Slattery CA, et al An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity. Mol Med 2007; 13(1–2):59–68PubMedGoogle Scholar
  2. Bingham A, Mamyrova G, Rother KI, et al Predictors of acquired lip-odystrophy in juvenile-onset dermatomyositis and a gradient of severity. Medicine (Baltimore) 2008; 87(2):70–86CrossRefGoogle Scholar
  3. Bitnum S, Daeschner CW, Jr., Travis LB et al Dermatomyositis. J Pediatr 1964; 64:101–31PubMedCrossRefGoogle Scholar
  4. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975a 292(7):344–347CrossRefGoogle Scholar
  5. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975b; 292(8):403–407CrossRefGoogle Scholar
  6. Brown VE, Pilkington CA, Feldman BM, Davidson JE. An international consensus survey of the diagnostic criteria for juvenile dermatomyo-sitis (JDM). Rheumatology (Oxford) 2006; 45(8):990–993CrossRefGoogle Scholar
  7. Christen-Zäch S, Seshadri R, Sundberg J et al Pathological nailfold cap-illaroscopy changes in juvenile dermatomyositis: association with duration of untreated disease, skin involvement over 36 months and a non-unicyclic disease course. Arthritis Rheum 2008; 58:571–576CrossRefGoogle Scholar
  8. Feldman BM, Ravelli A. Maintaining flexibility: Joints and other considerations. In: LG Rider, LM Pachman, FW Miller, H Bollar (eds) Myositis and you: A guide to juvenile dermatomyositis for patients, families and health care providers. The Myositis Association; 2007:269–274Google Scholar
  9. Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermato-myositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008; 371(9631):2201–2212PubMedCrossRefGoogle Scholar
  10. Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 2002; 47(4):505–511PubMedCrossRefGoogle Scholar
  11. Griffin TA, Reed AM. Pathogenesis of myositis in children. Curr Opin Rheumatol 2007; 19(5):487–491PubMedCrossRefGoogle Scholar
  12. Gunawardena H, Wedderburn LR, North J, et al Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile der-matomyositis. Rheumatology (Oxford) 2008; 47(3):324–328CrossRefGoogle Scholar
  13. Guzman J, Petty RE, Malleson PN. Monitoring disease activity in juvenile dermatomyositis: The role of von Willebrand factor and muscle enzymes. J Rheumatol 1994; 21:739–743PubMedGoogle Scholar
  14. Hazen PG, Walker AE, Carney JF, Stewart JJ. Cutaneous calcinosis of scleroderma. Successful treatment with intralesional adrenal steroids. Arch Dermatol 1982; 118(5):366–367PubMedCrossRefGoogle Scholar
  15. 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(3):541–549PubMedCrossRefGoogle Scholar
  16. Lindehammar H, Lindvall B. Muscle involvement in juvenile idiopathic arthritis Rheumatology (Oxford) 2004; 43(12):1546–1554CrossRefGoogle Scholar
  17. Lopez de Padilla CM, Vallejo AN, McNallan KT, et al Plasmacytoid dendritic cells in inflamed muscle of patients with juvenile dermato-myositis. Arthritis Rheum 2007; 56(5):1658–1668Google Scholar
  18. Mamyrova G., O'Hanlon TP, Sillers L, et al, for the Childhood Myositis Heterogeneity Collaborative Study Group. Cytokine gene polymorphisms as risk and severity factors for Juvenile Dermatomyositis. Arthritis Rheum 2008; 58(12):3941–50Google Scholar
  19. 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(10):1255–1260CrossRefGoogle Scholar
  20. 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(7):1183–1191PubMedCrossRefGoogle Scholar
  21. Miller, FW. Inflammatory myopathies: Polymyositis, dermatomyositis, and related conditions. In: Koopman W, Moreland L (eds) Arthritis and allied conditions — A textbook of rheumatology (15th edn, volume 2). Philadelphia, PA, Lippincott Williams & Wilkins, 2005, pp. 1593–1620Google Scholar
  22. Pachman LM, Hayford JR, Chung A et al Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. J Rheumatol 1998; 25:1198–1204PubMedGoogle Scholar
  23. Pachman LM, Abbott K, Sinacore JM, et al Duration of illness is an important variable for untreated children with juvenile dermatomy-ositis. J Pediatr 2006; 148(2):247–253PubMedCrossRefGoogle Scholar
  24. Pilkington C, Reed AM. When your child has more than one autoimmune disease. In: Rider LG, Pachman LM, Miller FW, Bollar H (eds) Myositis and you (1st edn), Addition. Washington DC, The Myositis Association, 2007, pp. 321–326Google Scholar
  25. Pilkington CA, Wedderburn LR. Paediatric idiopathic inflammatory muscle disease: Recognition and management. Drugs 2005; 65(10): 1355–1365PubMedCrossRefGoogle Scholar
  26. 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(11): 3570–3578PubMedCrossRefGoogle Scholar
  27. Reed AM, Lopez M. Juvenile dermatomyositis: Recognition and treatment. Paediatr Drugs 2002; 4(5):315–321PubMedGoogle Scholar
  28. Rider L, eed A, James-Newton L, Feldman B, Ravelli A, Cabalar I, Villalba M, Rider LG, Miller FW. New perspectives on the idio-pathic inflammatory myopathies of childhood. Curr Opin Rheumatol 1994; 6(6):575–582PubMedCrossRefGoogle Scholar
  29. Rider LG. Outcome assessment in the adult and juvenile idiopathic inflam-matory myopathies. Rheum Dis Clin North Am 2002; 28(4):935–977PubMedCrossRefGoogle Scholar
  30. Rider LG. The heterogeneity of juvenile myositis. Autoimmun Rev 2007; 6(4):241–247PubMedCrossRefGoogle Scholar
  31. Rider LG, Miller FW. Classification and treatment of the juvenile idio-pathic inflammatory myopathies. Rheum Dis Clin North Am 1997; 23(3):619–655PubMedCrossRefGoogle Scholar
  32. Riley P, McCann LJ, Maillard SM, Woo P, Murray KJ, Pilkington CA. Effectiveness of infliximab in the treatment of refractory juvenile der-matomyositis with calcinosis. Rheumatology 2008; 47(6): 877–880PubMedCrossRefGoogle Scholar
  33. Rouster-Stevens KA, Pachman LM. Autoantibody to signal recognition particle in African-American girls. J Rheumatol 35:927–929, 2008Google Scholar
  34. Sheshadri R, Feldman BM, Ilowite N, Cawkwell G, Pachman LM. The role of aggressive corticosteroid therapy in patients with juvenile dermatomyositis: A propensity score analysis. Arthritis Rheum 2008; 59(7):989–995CrossRefGoogle Scholar
  35. Targoff IN, Mamyrova G, Trieu EP, et al A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum 2006; 54(11):3682–3689PubMedCrossRefGoogle Scholar
  36. Wedderburn LR, McHugh NJ, Chinoy H, et al HLA class II haplotype and autoantibody associations in children with juvenile dermato-myositis and juvenile dermatomyositis-scleroderma overlap. Rheumatology (Oxford) 2007a; 46(12):1786–1791CrossRefGoogle Scholar
  37. Wedderburn LR, Varsani H, Li CK, et al International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis: A tool for potential use in clinical trials. Arthritis Rheum 2007b; 57(7):1192–1201CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • Ann M. Reed
    • 1
  • Clarissa A. Pilkington
    • 2
  • Brian M. Feldman
    • 3
  • Lauren M. Pachman
    • 4
  • Lisa G. Rider
    • 5
  1. 1.Mayo ClinicMinnesotaUSA
  2. 2.Great Ormond Street Rheumatology DepartmentUniversity CollegeLondonEngland
  3. 3.University of Toronto/SickKidsTorontoCanada
  4. 4.Department of Pediatrics, Division of RheumatologyChildren's Memorial Research Center, Children's Memorial Hospital, Northwestern University, Feinberg School of MedicineChicagoUSA
  5. 5.Environmental Autoimmunity Group National Institutes of Health / NIEHSBethesda

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