Zusammenfassung
Die juvenile Dermatomyositis (JDM) ist eine entzündliche Multisystemerkrankung unklarer Ätiologie mit besonderer Beteiligung der Haut und der quergestreiften Muskulatur. Nach einem Prodromalstadium tritt eine progressive proximale Muskelschwäche auf. An der Haut sind die klassischen Zeichen der heliotrope Ausschlag, Wangenerythem, Gottron-Zeichen und Nagelbettgefäßveränderungen. Zur Diagnose werden modifizierte Kriterien nach Bohan und Peter verwendet, die neben der klinischen Haut- und Muskelbeteiligung erhöhte Muskelenzymwerte und typische Befunde in Elektromyographie, Muskelbiopsie und neuerdings auch Magnetresonanztomographie (MRT) einbeziehen. Steroide werden klassisch hochdosiert oral gegeben. Eine intravenöse Pulstherapie mit niedriger dosierter oraler Intervalltherapie und weitere immunsuppressive Medikamente wie Methotrexat können Steroidnebenwirkungen vermindern. Die Prognose der JDM hat sich verbessert, und die meisten Patienten erholen sich, aber einige Patienten versterben und eine Minderheit hat Defektheilungen mit persistierender Muskelatrophie oder ausgeprägter Kalzinose.
Abstract
Juvenile dermatomyositis (JDM) is an inflammatory multi-system disease of unknown etiology with classic involvement of the skin and striated muscles. Following a prodromal period, patients develop a progressive proximal muscle weakness. Typical skin involvement includes heliotrope rash, facial erythema, Gottron’s sign and nailfold capillary abnormalities. For the diagnosis of JDM, modified Bohan and Peter criteria are used including clinical skin and muscle signs plus elevated muscle enzymes and typical findings from electromyography, muscle biopsy and – more recently – also on magnetic resonance imaging. Steroids are administered classically as high-dose oral treatment. Intravenous pulse therapy with intermittent lower dose oral treatment and other immunosuppressive drugs such as methotrexate may reduce steroid side-effects. Prognosis in JDM has improved, and most patients eventually make a full functional recovery. However, a few patients still die from their disease, and in a minority significant sequelae with muscle atrophy or severe calcinosis ensue.
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Literatur
Bode RK, Klein-Gitelman MS, Miller ML et al. (2003) Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence. Arthritis Rheum 49: 7–15
Brown VE, Pilkington CA, Feldman BM, Davidson JE (2006) An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford) 45: 990–993
Cassidy JT, Lindsley CB (2005) Juvenile dermatomyositis. In: Cassidy JT, Petty RE, Laxer RM, Lindsley CB (eds) Textbook of pediatric rheumatology, 5th edn. Elsevier Saunders, Philadelphia, pp 407–441
Crowe WE, Bove KE, Levinson JE et al. (1982) Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum 25: 126–139
Huber AM, Lang B, LeBlanc CMA et al. (2000) Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 43: 541–549
Huber AM, Feldman BM, Rennebohm RM 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: 1595–1603
Huppertz HI, Korinthenberg R (2005) Juvenile Dermatomyositis. In: Wahn U, Seger R, Wahn V, Holländer GA (Hrsg) Pädiatrische Allergologie und Immunologie, 4. Aufl. Urban & Fischer, München Jena, S 815–825
McCann LJ, Juggins AD, Maillard SM et al. (2006) The juvenile dermatomyositis national registry and repository (UK and Ireland)-clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford) 45: (advanced electronic publication March 27, 2006)
Mendez EP, Lipton R, Ramsey-Goldman R 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: 300–305
Naim MY, Reed AM (2006) Enzyme elevation in patients with juvenile dermatomyositis and steroid myopathy. J Rheumatol 33: 1392–1394
O’Connor KA, Abbott KA, Sabin B et al. (2006) MxA gene expression in juvenile dermatomyositis peripheral blood mononuclear cells: association with muscle involvement. Clin Immunol 120: 319–325
Oral EA, Simha V, Ruiz E et al. (2002) Leptin-replacement therapy for lipodystrophy. N Engl J Med 346: 570–578
Pachman LM, Liotta-Davis MR, Hong DK et al. (2000) TNF alpha-308A allele in juvenile dermatomyositis. Association with increased production of tumor necrosis factor alpha, disease duration, and pathologic calcifications. Arthritis Rheum 43: 2368–2377
Pachman LM, Abbott K, Sinacore JM et al. (2006) Duration of illness is an important variable for untreated children with juvenile dermatomyositis. J Pediatr 148: 247–253
Ramanan AV, Campbell-Webster N, Ota S et al. (2005) The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum 52: 3570–3578
Ravelli A, Ruperto N, Trail L et al. (2006) Clinical assessment in juvenile dermatomyositis. Autoimmunity 39: 197–203
Rider LG (2003) Calcinosis in juvenile dermatomyositis: pathogenesis and current therapies. Ped Rheumatol Online J 1:1 19–133 (Zugang im August 2006 unter http://www.pedrheumonlinejournal.org)
Ruperto N, Ravelli A, Murray KJ et al. (2003) Preliminary core set of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis. Rheumatology 42: 1452–1459
Symmons DP, Sills JA, Davis SM (1995) The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol 34: 732–736
Verma S, Singh S, Bhalla AK, Khullar M (2006) Study of subcutaneous fat in children with juvenile dermatomyositis. Arthritis Care Res 55: 564–568
Wargula JC, Lovell DJ, Passo MH et al. (2006) What more can we learn from muscle histopathology in children with dermatomyositis/polymyositis? Clin Exp Rheumatol 24: 333–343
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Dressler, F., Huppertz, HI. Juvenile Dermatomyositis. Z. Rheumatol. 65, 587–594 (2006). https://doi.org/10.1007/s00393-006-0109-5
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DOI: https://doi.org/10.1007/s00393-006-0109-5