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. 2018 Jun 25;16(1):40.
doi: 10.1186/s12969-018-0257-6.

Development of practice and consensus-based strategies including a treat-to-target approach for the management of moderate and severe juvenile dermatomyositis in Germany and Austria

Claas H Hinze  1 , Prasad T Oommen  2 , Frank Dressler  3 , Andreas Urban  4 , Frank Weller-Heinemann  5 , Fabian Speth  6   7 , Elke Lainka  8 , Jürgen Brunner  9 , Heike Fesq  10 , Dirk Foell  11 , Wolfgang Müller-Felber  12 , Ulrich Neudorf  8 , Christoph Rietschel  13 , Tobias Schwarz  14 , Ulrike Schara  15 , Johannes-Peter Haas  10
Affiliations

Development of practice and consensus-based strategies including a treat-to-target approach for the management of moderate and severe juvenile dermatomyositis in Germany and Austria

Claas H Hinze et al. Pediatr Rheumatol Online J. .

Abstract

Background: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous. The JDM working group of the Society for Pediatric Rheumatology (GKJR) aims to define consensus- and practice-based strategies in order to harmonize diagnosis, treatment and monitoring of JDM.

Methods: The JDM working group was established in 2015 consisting of 23 pediatric rheumatologists, pediatric neurologists and dermatologists with expertise in the management of JDM. Current practice patterns of management in JDM had previously been identified via an online survey among pediatric rheumatologists and neurologists. Using a consensus process consisting of online surveys and a face-to-face consensus conference statements were defined regarding the diagnosis, treatment and monitoring of JDM. During the conference consensus was achieved via nominal group technique. Voting took place using an electronic audience response system, and at least 80% consensus was required for individual statements.

Results: Overall 10 individual statements were developed, finally reaching a consensus of 92 to 100% regarding (1) establishing a diagnosis, (2) case definitions for the application of the strategies (moderate and severe JDM), (3) initial diagnostic testing, (4) monitoring and documentation, (5) treatment targets within the context of a treat-to-target strategy, (6) supportive therapies, (7) explicit definition of a treat-to-target strategy, (8) various glucocorticoid regimens, including intermittent intravenous methylprednisolone pulse and high-dose oral glucocorticoid therapies with tapering, (9) initial glucocorticoid-sparing therapy and (10) management of refractory disease.

Conclusion: Using a consensus process among JDM experts, statements regarding the management of JDM were defined. These statements and the strategies aid in the management of patients with moderate and severe JDM.

Keywords: Antirheumatic agents; Child; Comparative effectiveness research; Consensus; Dermatomyositis; Diagnosis.

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Conflict of interest statement

Ethics approval and consent to participate

The national pediatric rheumatology database was approved by the ethics committee of the Charité in Berlin.

Consent for publication

Not applicable.

Competing interests

Dr. Hinze has received consulting fees, speaking fees, and/or honoraria from Novartis (less than $10,000 each). Dr. Oommen reports no potential competing interests. Dr. Dressler has received consulting fees, speaking fees, and/or honoraria from Novartis and Pfizer (less than $10,000 each). Dr. Weller-Heinemann has received honoraria from Abbvie, Novartis and Pfizer (less than $10,000 each). Dr. Lainka has received consulting fees, speaking fees, and/or honoraria from Novartis (less than $10,000 each) and research support from Sobi. Dr. Brunner has received consulting fees, speaking fees, and/or honoraria from Abbvie, MSD, Novartis, Pfizer and Roche (less than $10,000 each). Dr. Föll is a board member of the Society for Pediatric Rheumatoloy and has received honoraria from Novartis, Pfizer, Roche-Chugai and Sobi (less than $10,000 each) and research support from Novartis and Pfizer. Dr. Neudorf has received consulting fees, speaking fees, and/or honoraria from Chugai and Philips (less than $10,000 each), and research support from Novartis. Dr. Schwarz reports no potential competing interests. Dr. Schara is president of the Gesellschaft für Neuropädiatrie (GNP; Society for Pediatric Neurology in Germany) and has received consulting fees, speaking fees, and/or honoraria from PTC Therapeutics, Deutsche Myasthenie Gesellschaft (German Myasthenia Society) and benni &co (a non-profit organization benifitting patients with muscular dystrophy). Dr. Haas is the president of the Society for Pediatric Rheumatology has received research support from Novartis and Pfizer.

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Figures

Fig. 1
Fig. 1
Treatment strategies in juvenile dermatomyositis (JDM). Patients with new-onset active JDM are treated with 1 of 3 glucocorticoid regimens and always receive an additional disease-modifying antirheumatic drug, preferably methotrexate with or without intravenous immune globulins. If the predefined treatment targets are achieved, glucocorticoids are tapered according to a scheduled outlined in Table 3. If treatment targets are not reached, a modification in therapy is needed, i.e. either addition of a new therapy or switching therapy. There was no consensus as to a specific sequence of changes in therapy. Patients may also be treated according to these strategies if they have pre-existing, active JDM (see right side of this figure). *Repeated cycles of intravenous methylprednisolone 20–30 mg/kg (max. 1000 mg) daily for 3–5 days in a row. **Prednisone equivalent 0.5–2 mg/kg (max. 80 mg) daily. †Prednisone equivalent 0.2–0.5 mg/kg daily. ‡Prednisone equivalent 2 mg/kg (max. 80 mg) daily. ¶Single cycle of intravenous methylprednisolone 20–30 mg/kg (max. 1000 mg) daily for 3–5 days. Abbreviations: AZA, azathioprin; CSA, cyclosporin A; CYC, cyclophosphamide; DMARD, disease-modifying antirheumatic drug; GC, glucocorticoids; IVIG, intravenous immune globulins; IVMP, intravenous methylprednisolone pulse; JDM, juvenile dermatomyositis; MTX, methotrexate; MMF, mycophenolate mofetil; RTX, rituximab; s.c., subcutaneously; TNFi, tumor necrosis factor inhibitor
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References

    1. Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet. 2008;371(9631):2201–2212. doi: 10.1016/S0140-6736(08)60955-1. - DOI - PubMed
    1. Brown VE, Pilkington CA, Feldman BM, Davidson JE, Network for Juvenile Dermatomyositis PRES An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM) Rheumatology (Oxford) 2006;45(8):990–993. doi: 10.1093/rheumatology/kel025. - DOI - PubMed
    1. Stringer E, Bohnsack J, Bowyer SL, Griffin TA, Huber AM, Lang B, Lindsley CB, Ota S, Pilkington C, Reed AM, 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(9):1953–1961. doi: 10.3899/jrheum.090953. - DOI - PubMed
    1. Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, Barohn RJ, Feldman BM, Harris-Love MO, Koontz DC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013;65(2):314–324. doi: 10.1002/art.37754. - DOI - PMC - PubMed
    1. Ruperto N, Pistorio A, Oliveira S, Zulian F, Cuttica R, Ravelli A, Fischbach M, Magnusson B, Sterba G, Avcin T, et al. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial. Lancet. 2016;387(10019):671–678. doi: 10.1016/S0140-6736(15)01021-1. - DOI - PubMed

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