Download PDF
Kinder- und Jugendmedizin 2015; 15(05): 343-350
DOI: 10.1055/s-0038-1629290
Kinder- und Jugendrheumatologie
Schattauer GmbH

Juvenile Dermatomyositis

Diagnose und TherapieJuvenile dermatomyositisDiagnosis and therapy
C. H. Hinze
1   Klinik für Pädiatrische Rheumatologie und Immunologie, Universitätsklinikum Münster
› Author Affiliations
Further Information

Publication History

Eingereicht am: 15 June 2015

angenommen am: 20 June 2015

Publication Date:
27 January 2018 (online)

    Permissions and Reprints

Zusammenfassung

Die juvenile Dermatomyositis ist die häufigste chronisch-entzündliche Myopathie des Kindesalters, charakterisiert durch Haut- und Gefäßveränderungen und Muskelschwäche, am häufigsten im frühen Schulalter auftre-tend. Die pathognomonische Befundkonstellation erlaubt prinzipiell eine frühe klinische Diagnose. Frühe Diagnose und frühe, aggressive pharmakologische, physiotherapeutische und supportive Therapien sind für die Prog-nose entscheidend. Bedeutsame Komplikationen sind Organbeteiligung, Ulzerationen, Kontrakturen, dystrophe Kalzifizierung und Lipodystrophie. Für die optimale Versorgung ist eine enge Zusammenarbeit zwischen Kinder- und Jugendarzt und Kinderrheumatologen unabdingbar. Das Erkennen der typischen Befundkonstellation und Kenntnis angemessener Therapieverfahren sind auch für den niedergelassenen Kinder- und Jugendarzt wichtig. Die typischen Befunde der klinischen Untersuchung, im Labor und in der Bildgebung, die Differenzialdiagnose, Komplikationen der Erkrankung und moderne Therapieansätze werden in diesem Übersichtsartikel diskutiert.

Summary

Juvenile dermatomyositis is the most common chronic inflammatory myopathy of childhood, characterized by vascular and skin findings and muscle weakness, with typical onset in early school age. Pathognomonic findings usually allow for early diagnosis. Early diagnosis and early, aggressive therapy, including drugs, physiotherapy and supportive therapies, are extremely important in order to avoid serious sequelae, such as organ involvement, ulcerations, joint contractures, dystrophic calcification and lipodys-trophy. Therefore, close collaboration between the primary care provider and pediatric rheumatologist is essential. Knowledge of typical findings in juvenile dermatomyositis and adequate therapies is important for the general pediatrician as well. This review discusses typical clinical, laboratory and imaging findings in juvenile dermatomyositis, as well as its differential diagnosis, complications and modern therapies.

Schlüsselwörter

Juvenile Dermatomyositis - Vaskulopathie

Keywords

Juvenile dermatomyositis - vasculopathy
 

  • Literatur

  • 1 Feldman BM. et al. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008; 371: 2201-2212.
    CrossrefPubMedGoogle Scholar
  • 2 Miller FW. et al. Genome-wide association study of dermatomyositis reveals genetic overlap with other autoimmune disorders. Arthritis Rheum 2013; 65: 3239-3247.
    CrossrefPubMedGoogle Scholar
  • 3 Mamyrova G. et al. Immunogenetic risk and protective factors for juvenile dermatomyositis in Caucasians. Arthritis Rheum 2006; 54: 3979-3987.
    CrossrefPubMedGoogle Scholar
  • 4 Shah M. et al. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013; 92: 25-41.
    CrossrefPubMedGoogle Scholar
  • 5 Baechler EC, Bilgic H, Reed AM. Type I interferon pathway in adult and juvenile dermatomyositis. Arthritis Res Ther 2011; 13: 249
    CrossrefPubMedGoogle Scholar
  • 6 Rider LG. et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013; 92: 223-43.
    CrossrefPubMedGoogle Scholar
  • 7 Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975; 292: 344-347.
    CrossrefPubMedGoogle Scholar
  • 8 Robinson AB. et al. Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry. Arthritis Care Res (Hoboken) 2014; 66: 404-410.
    CrossrefPubMedGoogle Scholar
  • 9 Mamyrova G. et al. Clinical and laboratory features distinguishing juvenile polymyositis and muscular dystrophy. Arthritis Care Res (Hoboken) 2013; 65: 1969-1975.
    CrossrefPubMedGoogle Scholar
  • 10 Bingham A. et al. Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity. Medicine (Baltimore) 2008; 87: 70-86.
    CrossrefPubMedGoogle Scholar
  • 11 Abinun M. et al. Heterogeneity of juvenile dermatomyositis. Lancet 2000; 355: 1186
    PubMedGoogle Scholar
  • 12 Rider LG. et al. Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res (Hoboken) 2011; 63 (Suppl. 11) Suppl S118-157.
    CrossrefPubMedGoogle Scholar
  • 13 Disease Activity Core Set Measures 2014. Available from. www.niehs.nih.gov/research/resources/imacs/diseaseactivity/index.cfm
    Google Scholar
  • 14 Tomasova Studynkova J. et al. The role of MRI in the assessment of polymyositis and dermatomyositis. Rheumatology (Oxford) 2007; 46: 1174-1179.
    CrossrefPubMedGoogle Scholar
  • 15 Miles L. 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: 1183-1191.
    CrossrefPubMedGoogle Scholar
  • 16 Pachman LM. et al. Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. J Rheumatol 1998; 25: 1198-1204.
    Google Scholar
  • 17 Brown VE. et al. An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford) 2006; 45: 990-993.
    CrossrefPubMedGoogle Scholar
  • 18 Ladd PE. et al. Juvenile dermatomyositis: correlation of MRI at presentation with clinical outcome. AJR Am J Roentgenol 2011; 197: W153-158.
    CrossrefPubMedGoogle Scholar
  • 19 Schmeling H. et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford) 2011; 50: 885-893.
    CrossrefPubMedGoogle Scholar
  • 20 Bloom BJ. et al. von Willebrand factor in juvenile dermatomyositis. J Rheumatol 1995; 22: 320-325.
    Google Scholar
  • 21 Rider LG. et al. Neopterin and quinolinic acid are surrogate measures of disease activity in the juvenile idiopathic inflammatory myopathies. Clin Chem 2002; 48: 1681-1688.
    PubMedGoogle Scholar
  • 22 Miller FW. et al. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology (Oxford) 2001; 40: 1262-1273.
    CrossrefPubMedGoogle Scholar
  • 23 Ravelli A. et al. Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res (Hoboken) 2010; 62: 63-72.
    CrossrefPubMedGoogle Scholar
  • 24 Tse S. et al. The arthritis of inflammatory childhood myositis syndromes. J Rheumatol 2001; 28: 192-197.
    Google Scholar
  • 25 Wedgwood RJ, Cook CD, Cohen J. Dermatomyositis; report of 26 cases in children with a discussion of endocrine therapy in 13. Pediatrics 1953; 12: 447-466.
    PubMedGoogle Scholar
  • 26 Bowyer SL. et al. Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr 1983; 103: 882-888.
    CrossrefPubMedGoogle Scholar
  • 27 Stringer E, Singh-Grewal D, Feldman BM. Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis Rheum 2008; 58: 3585-3592.
    CrossrefPubMedGoogle Scholar
  • 28 Lam CG. et al. Efficacy of intravenous Ig therapy in juvenile dermatomyositis. Ann Rheum Dis 2011; 70: 2089-2094.
    CrossrefPubMedGoogle Scholar
  • 29 Huber AM. et al. Consensus treatments for moderate juvenile dermatomyositis: beyond the first two months. Results of the second Childhood Arthritis and Rheumatology Research Alliance consensus conference. Arthritis Care Res (Hoboken) 2012; 64: 546-553.
    CrossrefPubMedGoogle Scholar
  • 30 Lang B, Dooley J. Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis. J Pediatr 1996; 128: 429-432.
    CrossrefPubMedGoogle Scholar
  • 31 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: 360-368.
    CrossrefPubMedGoogle Scholar
  • 32 Dressler F, Huppertz HI. Juvenile dermatomyositis. Z Rheumatol 2006; 65: 587-590. 592-594
    CrossrefPubMedGoogle Scholar
  • 33 Ramanan AV. et al. The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum 2005; 52: 3570-3578.
    CrossrefPubMedGoogle Scholar
  • 34 Rouster-Stevens KA. et al. Mycophenolate mofetil: a possible therapeutic agent for children with juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62: 1446-1451.
    CrossrefPubMedGoogle Scholar
  • 35 Ruperto N. et al. A randomized trial in new onset juvenile dermatomyositis: Prednisone versus prednisone plus cyclosporine versus prednisone plus methotrexate, in ACR Meeting. 2012
    PubMedGoogle Scholar
  • 36 Aggarwal R. et al. Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis. Arthritis Rheumatol 2014; 66: 740-749.
    CrossrefPubMedGoogle Scholar
  • 37 Speth F, Hinze C, Haas JP. A prospective single-centre study on indicators of infectious risk after rituximab therapy in children and adolescents with rheumatic diseases, in Annual European Congress of Rheumatology EULAR 2015. Rom: 2015
    Google Scholar
  • 38 Rouster-Stevens KA. et al. Pilot study of etanercept in patients with refractory juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2014; 66: 783-787.
    CrossrefPubMedGoogle Scholar
  • 39 Robinson AB, Reed AM. Clinical features, pathogenesis and treatment of juvenile and adult dermatomyositis. Nat Rev Rheumatol 2011; 7: 664-675.
    CrossrefPubMedGoogle Scholar
  • 40 Olson NY, Lindsley CB. Adjunctive Use of Hydroxychloroquine in Childhood Dermatomyositis. Journal of Rheumatology 1989; 16: 1545-1547.
    PubMedGoogle Scholar
  • 41 Robinson AB. et al. Disease activity, proteinuria, and vitamin D status in children with systemic lupus erythematosus and juvenile dermatomyositis. J Pediatr 2012; 160: 297-302.
    CrossrefPubMedGoogle Scholar
  • 42 Maillard SM. et al. Quantitative assessments of the effects of a single exercise session on muscles in juvenile dermatomyositis. Arthritis Rheum 2005; 53: 558-564.
    CrossrefPubMedGoogle Scholar
  • 43 Omori CH. et al. Exercise training in juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2012; 64: 1186-1194.
    Google Scholar