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Medicine (Baltimore). 2013 Jan;92(1):25-41. doi: 10.1097/MD.0b013e31827f264d.

The clinical phenotypes of the juvenile idiopathic inflammatory myopathies.

Collaborators (142)

Abramson LS, Adams B, Albert DA, Amoroso K, Arabshahi B, Arthur ER, Athreya BH, Baer AN, Balboni IM, Bingham C, Blocker WP, Bohnsack JF, Ballinger S, Boire G, Borzy M, Botstein GR, Bowyer S, Burnham JM, Carrasco R, Cartwright VW, Cawkwell GD, Chao CP, Crisp E, Cron RQ, DeGuzman MM, Dietz MH, Eberhart A, Edelheit BS, Eggert JF, Eichenfield AH, Elder ME, Ellsworth JE, Fearn KA, Finkel TH, Flato I, Fuhlbrigge RC, Gabriel CA, Garwood VF, Gedalia A, Gehringer NL, George SW, Gewanter HL, Goldmuntz EA, Goldsmith DP, Gorden P, Gordon GV, Gospodinoff AC, Gottlieb B, Griffin TA, Groh BP, Haftel HM, Hawkins-Holt M, Henrickson M, Higgins GC, Ho G, Hoeltzel MF, Hollister J, Hopp RJ, Ilowite NT, Imundo L, Jacobs JC, James-Newton L, Jansen A, Jarvis J, Jerath R, Johnson CR, Jones OY, Jung LK, Kagen LJ, Kantor TV, Katona IM, Katz JD, Kimura Y, Kingsbury DJ, Klein SJ, Knee C, Knibbe W, Kurahara DK, Lang BA, Lasky A, Lawton AR, Lee J, Levine J, Lindsley CB, Lipnick RN, Lourie SH, Madson KL, Marks HG, McCarthy PL, Miller JJ 3rd, Mitchell SR, Moallem HJ, Morishima C, Murphy FT, O'Hanlon T, Oen KG, Olson JC, Oral EA, Ostrov BE, Pachman LM, Pappu R, Passo MH, Perez MD, Person DA, Peterson KS, Plotz PH, Punaro MG, Rabinovich C, Radis CD, Ray LI, Reed AM, Rennebohm RM, Reuman PD, Rivas-Chacon RF, Rothman D, Schikler KN, Scott DW, Shaham B, Sheets RM, Sherry DD, Sills E, Sinal SH, Smukler A, Starr AJ, Sule SH, Sundel RP, Szer IS, Taylor SI, Taylor-Albert ES, Vehe RK, Vogelgesang SA, Vogler LB, Von Scheven E, Wall S, Wallace CA, Wargula JC, White PH, Wilkenfeld M, Wilking AP, Wu L, Yung CM, Zemel LS.

Author information

Environmental Autoimmunity Group, Program of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, DHHS, Bethesda, Maryland 20892-1301, USA.


The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features were shared between juvenile and adult IIM subgroups. However, JDM and JPM patients had a lower frequency of interstitial lung disease, Raynaud phenomenon, "mechanic's hands" and carpal tunnel syndrome, and lower mortality than their adult counterparts. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct clinical subgroups, defined by varying clinical and demographic characteristics, laboratory features, and outcomes.

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