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

Overview

Journal Pediatr Rheumatol Online J

Publisher Biomed Central

Specialty Pediatrics

Date 2018 Jun 27

PMID 29940960

Citations 15

Authors

Claas H Hinze

Prasad T Oommen

Frank Dressler

Andreas Urban

Frank Weller-Heinemann

Fabian Speth

Elke Lainka

Jurgen Brunner

Heike Fesq

Dirk Foell

Wolfgang Muller-Felber

Ulrich Neudorf

Christoph Rietschel

Tobias Schwarz

Ulrike Schara

Johannes-Peter Haas

Affiliations

Soon will be listed here.

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.

Citing Articles

Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.

Kim H Paediatr Drugs. 2024; 27(1):57-72.

PMID: 39425894 PMC: 11774970. DOI: 10.1007/s40272-024-00658-2.

TIF1-gamma IgG2 isotype is not associated with malignancy in juvenile dermatomyositis patients.

Nguyen H, Jouen F, Dechelotte B, Cordel N, Gitiaux C, Bodemer C Rheumatology (Oxford). 2024; 63(10):e281-e284.

PMID: 38498839 PMC: 11443014. DOI: 10.1093/rheumatology/keae182.

[Advances in the diagnostics and treatment of juvenile dermatomyositis].

Georgi I, Georgi M, Haas J Z Rheumatol. 2023; 83(1):41-51.

PMID: 38157051 DOI: 10.1007/s00393-023-01454-y.

Treat-to-target strategies for the management of familial Mediterranean Fever in children.

Ehlers L, Rolfes E, Lieber M, Muller D, Lainka E, Gohar F Pediatr Rheumatol Online J. 2023; 21(1):108.

PMID: 37752496 PMC: 10521475. DOI: 10.1186/s12969-023-00875-y.

Rapid and sustained response to JAK inhibition in a child with severe MDA5 + juvenile dermatomyositis.

Strauss T, Gunther C, Schnabel A, Wolf C, Hahn G, Lee-Kirsch M Pediatr Rheumatol Online J. 2023; 21(1):104.

PMID: 37726751 PMC: 10507825. DOI: 10.1186/s12969-023-00894-9.

References

Hinze C, Speth F, Oommen P, Haas J . Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists. Pediatr Rheumatol Online J. 2018; 16(1):38. PMC: 6011340. DOI: 10.1186/s12969-018-0256-7. View

Stoffer M, Schoels M, Smolen J, Aletaha D, Breedveld F, Burmester G . Evidence for treating rheumatoid arthritis to target: results of a systematic literature search update. Ann Rheum Dis. 2015; 75(1):16-22. PMC: 4717391. DOI: 10.1136/annrheumdis-2015-207526. View

Huber A, Robinson A, Reed A, Abramson L, Bout-Tabaku S, Carrasco R . 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). 2011; 64(4):546-53. PMC: 3315594. DOI: 10.1002/acr.20695. View

Morgan DeWitt E, Brunner H . The landscape of comparative effectiveness research in rheumatology. Nat Rev Rheumatol. 2013; 10(1):57-62. DOI: 10.1038/nrrheum.2013.140. View

Crowe W, Bove K, LEVINSON J, Hilton P . Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum. 1982; 25(2):126-39. DOI: 10.1002/art.1780250203. View

Rouster-Stevens K, Gursahaney A, Ngai K, Daru J, Pachman L . Pharmacokinetic study of oral prednisolone compared with intravenous methylprednisolone in patients with juvenile dermatomyositis. Arthritis Rheum. 2008; 59(2):222-6. PMC: 2852143. DOI: 10.1002/art.23341. View

McCann L, Kirkham J, Wedderburn L, Pilkington C, Huber A, Ravelli A . Development of an internationally agreed minimal dataset for juvenile dermatomyositis (JDM) for clinical and research use. Trials. 2015; 16:268. PMC: 4472260. DOI: 10.1186/s13063-015-0784-0. View

Hinze C, Gohar F, Foell D . Management of juvenile idiopathic arthritis: hitting the target. Nat Rev Rheumatol. 2015; 11(5):290-300. DOI: 10.1038/nrrheum.2014.212. View

Gunawardena H, Wedderburn L, North J, Betteridge Z, Dunphy J, Chinoy H . Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology (Oxford). 2008; 47(3):324-8. DOI: 10.1093/rheumatology/kem359. View

10.

Ruperto N, Pistorio A, Oliveira S, Zulian F, Cuttica R, Ravelli A . Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial. Lancet. 2015; 387(10019):671-678. DOI: 10.1016/S0140-6736(15)01021-1. View

11.

Spencer C, Rouster-Stevens K, Gewanter H, Syverson G, Modica R, Schmidt K . Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America. Pediatr Rheumatol Online J. 2017; 15(1):50. PMC: 5470177. DOI: 10.1186/s12969-017-0174-0. View

12.

Rider L, Nistala K . The juvenile idiopathic inflammatory myopathies: pathogenesis, clinical and autoantibody phenotypes, and outcomes. J Intern Med. 2016; 280(1):24-38. PMC: 4914449. DOI: 10.1111/joim.12444. View

13.

Dressler F, Huppertz H . [Juvenile dermatomyositis]. Z Rheumatol. 2006; 65(7):587-90, 592-4. DOI: 10.1007/s00393-006-0109-5. View

14.

Rouster-Stevens K, Morgan G, Wang D, Pachman L . Mycophenolate mofetil: a possible therapeutic agent for children with juvenile dermatomyositis. Arthritis Care Res (Hoboken). 2010; 62(10):1446-51. PMC: 2952049. DOI: 10.1002/acr.20269. View

15.

Ravelli A, Trail L, Ferrari C, Ruperto N, Pistorio A, Pilkington C . Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res (Hoboken). 2010; 62(1):63-72. DOI: 10.1002/acr.20015. View

16.

Tiniakou E, Pinal-Fernandez I, Lloyd T, Albayda J, Paik J, Werner J . More severe disease and slower recovery in younger patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Rheumatology (Oxford). 2017; 56(5):787-794. PMC: 5850825. DOI: 10.1093/rheumatology/kew470. View

17.

Stringer E, Bohnsack J, Bowyer S, Griffin T, Huber A, Lang B . 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):1953-61. DOI: 10.3899/jrheum.090953. View

18.

Oddis C, Reed A, Aggarwal R, Rider L, Ascherman D, Levesque M . Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2012; 65(2):314-24. PMC: 3558563. DOI: 10.1002/art.37754. View

19.

Rider L, Shah M, Mamyrova G, Huber A, Rice M, Targoff I . The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore). 2013; 92(4):223-243. PMC: 3721421. DOI: 10.1097/MD.0b013e31829d08f9. View

20.

Tansley S, Betteridge Z, Simou S, Jacques T, Pilkington C, Wood M . Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients. J Rheumatol. 2017; 44(4):488-492. DOI: 10.3899/jrheum.160871. View