https://doi.org/10.4081/reumatismo.2024.1640
Predictors of poor outcomes in juvenile dermatomyositis: what do we know? A narrative review
Submitted: 27 August 2023
Accepted: 19 June 2024
Published: 11 November 2024
Accepted: 19 June 2024
Abstract Views: 75
PDF: 20
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Objective. Juvenile dermatomyositis (JDM) is a rare chronic systemic inflammatory disorder with a highly variable clinical course. It is important to identify the patients at risk of developing more severe disease. However, based on the existing literature, there is a lack of data regarding predictors of poor outcomes. Obtaining knowledge about clinical and laboratory risk factors for disease progression and severity at an earlier stage of the disease could potentially lead to a better long-term prognosis for patients with JDM.
Methods. A narrative review with the aim of identifying risk factors for poor outcomes in patients with JDM, such as death, severe disease, refractory disease, and functional impairment, was conducted. A total of 27 articles was included.
Results. Certain clinical manifestations and immunology features appear to worsen the prognosis in children with JDM. The recognition of these risk factors is essential for all pediatric rheumatologists as it allows the earlier identification of patients with potentially worse outcomes. These patients should receive closer follow-up and aggressive and individualized therapy in order to reduce their morbimortality.
Conclusions. Additional research is needed not only to identify more predictors of worse outcomes but also to identify more effective treatment approaches targeted toward these patients.
Huber AM. Juvenile idiopathic inflammatory myopathies. Pediatr Clin North Am 2018; 65: 739-56.
DOI: https://doi.org/10.1016/j.pcl.2018.04.006
Symmons DP, Sills JA, Davis SM. The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol 1995; 34: 732-6.
DOI: https://doi.org/10.1093/rheumatology/34.8.732
Huber A, Feldman BM. Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going? Curr Rheumatol Rep 2005; 7: 441-6.
DOI: https://doi.org/10.1007/s11926-005-0048-1
Huber AM, Mamyrova G, Lachenbruch PA, Lee JA, Katz JD, Targoff IN, et al. Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies. Arthritis Care Res 2014; 66: 732-40.
DOI: https://doi.org/10.1002/acr.22212
Ravelli A, Trail L, Ferrari C, Ruperto N, Pistorio A, Pilkington 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.
DOI: https://doi.org/10.1002/acr.20015
Sanner H, Sjaastad I, Flato B. Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool. Rheumatology 2014; 53: 1578-85.
DOI: https://doi.org/10.1093/rheumatology/keu146
Kendall FP ME, Provance PG. Muscles: testing and function. 4th edition. Third edition ed: Baltimore: Williams & Wilkins; 1993.
Lovell DJ, Lindsley CB, Rennebohm RM, Ballinger SH, Bowyer SL, Giannini EH, et al. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum 1999; 42: 2213-9.
DOI: https://doi.org/10.1002/1529-0131(199910)42:10<2213::AID-ANR25>3.0.CO;2-8
Isenberg DA, Allen E, Farewell V, Ehrenstein MR, Hanna MG, Lundberg IE, et al. International consensus outcome measures for patients with idiopathic inflammatory myopathies. Development and initial validation of myositis activity and damage indices in patients with adult onset disease. Rheumatology 2004; 43: 49-54.
DOI: https://doi.org/10.1093/rheumatology/keg427
Bode RK, Klein-Gitelman MS, Miller ML, Lechman TS, Pachman LM. Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence. Arthritis Rheum 2003; 49: 7-15.
DOI: https://doi.org/10.1002/art.10924
Stringer E, Singh-Grewal D, Feldman BM. Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis Rheum 2008; 58: 3585-92.
DOI: https://doi.org/10.1002/art.23960
Spencer-Green G, Schlesinger M, Bove KE, Levinson JE, Schaller JG, Hanson V, et al. Nailfold capillary abnormalities in childhood rheumatic diseases. J Pediatr 1983; 102: 341-6.
DOI: https://doi.org/10.1016/S0022-3476(83)80645-3
Smith RL, Sundberg J, Shamiyah E, Dyer A, Pachman LM. Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. J Rheumatol 2004;3 1: 1644-9.
Christen-Zaech S, Seshadri R, Sundberg J, Paller AS, Pachman LM. Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis. Arthritis Rheum 2008; 58: 571-6.
DOI: https://doi.org/10.1002/art.23299
Schmeling H, Stephens S, Goia C, Manlhiot C, Schneider R, Luthra S, et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology 2011; 50: 885-93.
DOI: https://doi.org/10.1093/rheumatology/keq407
Pachman LM, Nolan BE, DeRanieri D, Khojah AM. Juvenile dermatomyositis: new clues to diagnosis and therapy. Curr Treatm Opt Rheumatol 2021; 7: 39-62.
DOI: https://doi.org/10.1007/s40674-020-00168-5
Tansley SL, Simou S, Shaddick G, Betteridge ZE, Almeida B, Gunawardena H, et al. Autoantibodies in juvenile-onset myositis: their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun 2017; 84: 55-64.
DOI: https://doi.org/10.1016/j.jaut.2017.06.007
Rider LG, Shah M, Mamyrova G, Huber AM, Rice MM, Targoff IN, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine 2013; 92: 223-43.
DOI: https://doi.org/10.1097/MD.0b013e31829d08f9
Sabbagh S, Pinal-Fernandez I, Kishi T, Targoff IN, Miller FW, Rider LG, et al. Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis. Ann Rheum Dis 2019; 78: 988-95.
DOI: https://doi.org/10.1136/annrheumdis-2018-215004
Kishi T, Warren-Hicks W, Bayat N, Targoff IN, Huber AM, Ward MM, et al. Corticosteroid discontinuation, complete clinical response and remission in juvenile dermatomyositis. Rheumatology 2021; 60: 2134-45.
DOI: https://doi.org/10.1093/rheumatology/keaa371
Deakin CT, Yasin SA, Simou S, Arnold KA, Tansley SL, Betteridge ZE, et al. Muscle biopsy findings in combination with myositis-specific autoantibodies aid prediction of outcomes in juvenile dermatomyositis. Arthritis Rheumatol 2016; 68: 2806-16.
DOI: https://doi.org/10.1002/art.39753
Ladd PE, Emery KH, Salisbury SR, Laor T, Lovell DJ, Bove KE. Juvenile dermatomyositis: correlation of MRI at presentation with clinical outcome. AJR Am J Roentgenol 2011; 197: W153-8.
DOI: https://doi.org/10.2214/AJR.10.5337
Tansley SL, Betteridge ZE, Gunawardena H, Jacques TS, Owens CM, Pilkington C, et al. Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study. Arthritis Res Ther 2014; 16: R138.
DOI: https://doi.org/10.1186/ar4600
Barth Z, Schwartz T, Flato B, Aalokken TM, Koller A, Lund MB, et al. Association between nailfold capillary density and pulmonary and cardiac involvement in medium to longstanding juvenile dermatomyositis. Arthritis Care Res 2019; 71: 492-7.
DOI: https://doi.org/10.1002/acr.23687
Xu Y, Ma X, Zhou Z, Li J, Hou J, Zhu J, et al. Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature. Pediatr Rheumatol Online J 2021; 19: 2.
DOI: https://doi.org/10.1186/s12969-020-00486-x
Wakiguchi H, Takei S, Imanaka H, Hiraki T, Higashi M, Yamatou T, et al. Severe gluteal skin ulcers in an infant with juvenile dermatomyositis. Eur J Dermatol 2016; 26: 192-3.
DOI: https://doi.org/10.1684/ejd.2015.2701
Papadopoulou C, McCann LJ. The vasculopathy of juvenile dermatomyositis. Front Pediatr 2018; 6: 284.
DOI: https://doi.org/10.3389/fped.2018.00284
Chung MP, Richardson C, Kirakossian D, Orandi AB, Saketkoo LA, Rider LG, et al. Calcinosis biomarkers in adult and juvenile dermatomyositis. Autoimmun Rev 2020; 19: 102533.
DOI: https://doi.org/10.1016/j.autrev.2020.102533
Tsaltskan V, Aldous A, Serafi S, Yakovleva A, Sami H, Mamyrova G, et al. Long-term outcomes in juvenile myositis patients. Semin Arthritis Rheum 2020; 50: 149-55.
DOI: https://doi.org/10.1016/j.semarthrit.2019.06.014
Tansley SL, Betteridge ZE, Shaddick G, Gunawardena H, Arnold K, Wedderburn LR, et al. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology 2014; 53: 2204-8.
DOI: https://doi.org/10.1093/rheumatology/keu259
Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 2002; 47: 505-11.
DOI: https://doi.org/10.1067/mjd.2002.122196
Bingham A, Mamyrova G, Rother KI, Oral E, Cochran E, Premkumar A, et al. Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity. Medicine 2008; 87: 70-86.
DOI: https://doi.org/10.1097/MD.0b013e31816bc604
Bowyer SL, Blane CE, Sullivan DB, Cassidy JT. Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr 1983; 103: 882-8.
DOI: https://doi.org/10.1016/S0022-3476(83)80706-9
Huber AM, Lang B, LeBlanc CM, Birdi N, Bolaria RK, Malleson P, et al. Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 2000; 43: 541-9.
DOI: https://doi.org/10.1002/1529-0131(200003)43:3<541::AID-ANR9>3.0.CO;2-T
How to Cite
Martins, A., Ganhão, S., Aguiar, F., Rodrigues, M., & Brito, I. (2024). Predictors of poor outcomes in juvenile dermatomyositis: what do we know? A narrative review. Reumatismo. https://doi.org/10.4081/reumatismo.2024.1640
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