Results of the German ESPED-Recording of New Patients with Juvenile Dermatomyositis (JDM)

Abstract

In a nation-wide registration project 38 incident cases of juvenile dermatomyositis were collected in Germany over a 2-year-period. Diagnostic methods as well as the primary treatment for these patients were recorded. Detailed information was available for 25 of these patients. Diagnostias well as therapeutic decisions varied widely. Steroids were used in almost all of the 25 patients either as oral or as parenteral pulse therapy, additional immunosuppressive drugs were used in 52%. We plan to establish national consensus recommendations for diagnostic and therapeutic standards in JDM. Due to the rarity of JDM clinical trials will have to be performed on an international basis.

Zusammenfassung

In einer nationalen deutschen ESPED-Erhebung wurden 38 neue Fälle von juveniler Dermatomyositis (JDM) in 2 Jahren gemeldet. Die diagnostischen und initialen therapeutischen Schritte wurden per Fragebögen erfasst. Detaillierte Angaben lagen zu 25 Patienten vor. Die diagnostischen und therapeutischen Maßnahmen unterschieden sich deutlich. Steroide wurden bei fast allen der 25 Patienten oral oder parenteral in Pulsen eingesetzt, eine zusätzliche medikamentöse Immunsuppression erfolgte bei 52%. Wir planen nationale Konsensusleitlinien für diagnostische und therapeutische Standards für die JDM zu erstellen. Aufgrund der Seltenheit der Erkrankung müssen klinische Studien international erfolgen.

Key words

juvenile - dermatomyositis - diagnosis - therapy

Schlüsselwörter

juvenile - Dermatomyositis - Diagnose - Therapie -

References

• 1 Bohan A, Peter JB. Polymyositis and dermatomyositis (two parts).  N Engl J Med. 1975;  292 344-347 and 403–407
• 2 Brown VE, Pilkington CA, Feldman BM. et al . An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM).  Rheumatol. 2006;  45 990-993
• 3 Cooper MA, Willingham DL, Brown DE. et al . Rituximab for the treatment of juvenile dermatomyositis. A report of four pediatric patients.  Arthritis Rheum. 2006;  56 3107-3111
• 4 Dressler F, Huppertz HI. Juvenile Dermatomyositis.  Z Rheumatol. 2006;  65 587-594
• 5 Göbel U, Kontny U, Gortner L. et al . Networking in pediatrics and the journal's view.  Klin Padiatr. 2009;  150 131-133
• 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. 2010;  62 219-225
• 7 Huppertz HI, Frosch M, Martin C. et al . JDM-Studiengruppe: Treatment of juvenile dermatomyositis with high-dose oral steroids or with steroid pulses and low-dose oral steroids.  Ann Rheum Dis. 2000;  59 728
• 8 Levy DM, Bingham CA, Kahn PJ. et al . Favorable outcome of juvenile dermatomyositis treated without systemic corticosteroids.  J Pediatr. 2010;  156 302-307
• 9 Martin N, Krol P, Smith S. et al . A national registry for juvenile dermatomyositis and other pediatric inflammatory myopathies: 10 years’ experience; the Juvenile Dermatomyositis National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies.  Rheumatology. 2011;  50 137-145
• 10 Ramanan AV, Campbell-Webster N, Ota S. et al . The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered steroids.  Arthritis Rheum. 2005;  52 3570-3578
• 11 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. 2010;  62 63-72
• 12 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;  59 222-226
• 13 Rouster-Stevens KA, Morgan GA, Wang D. et al . Mycophenolate mofetil: A possible therapeutic agent for children with juvenile dermatomyositis.  Arthritis Care Res. 2010;  62 1446-1451
• 14 Ruperto N, Ravelli A, Pistorio A. et al . The provisional Paediatric Rheumatology Trials Organisation/American College of Rheumatology/European League against Rheumatism disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: A prospective validation study.  Arthritis Rheum. 2008;  59 4-13
• 15 Sanner H, Gran JT, Sjaastad I. et al . Cumulative organ damage and prognostic factors in juvenile dermatomyositis: A cross-sectional study median 16.8 years after symptom onset.  Rheumatol. 2009;  48 1541-1547
• 16 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;  59 989-995
• 17 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 1953-1961

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