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Medicine (Baltimore). 2013 Jul;92(4):223-43. doi: 10.1097/MD.0b013e31829d08f9.

The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

Collaborators (132)

Abramson LS, Adams B, Albert DA, Amoroso K, Arabshahi B, Arthur ER, Athreya BH, Baer AN, Balboni AN, Ballinger SH, Bingham CA, Blocker WP, Bohnsack JF, Boire G, Borzy MS, Botstein GR, Bowyer S, Burnham JM, Carrasco R, Cartwright VW, Cawkell GD, Chao CP, Cron RQ, DeGuzman MM, Eberhart BA, Edelheit BS, Eggert JF, Eichenfield AH, Elder ME, Ellsworth JE, Finkel TH, Flatau 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 BS, Griffin TA, Groh BP, Haftel HM, Hawkins-Holt M, Henrickson M, Higgins GC, Ho G Jr, Hoeltzel MF, Hollister JR, Hopp RJ, Ilowite NT, Imundo L, Jacobs JC, James-Newton L, Jansen A, Jarvis J, Jerath RS, Johnson CR, Jones OY, Jung LK, Kantor TV, Katona IM, Katz JD, Kimura Y, Kingsbury DJ, Klein SJ, Knee CM, Knibbe WP, Kurahara DK, Lang BA, Lasky A, Lawton AR, Lee JA, Levine J, Lindsley CB, 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 CE, 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 EM, Sinal SH, Smukler A, Sule SD, Sundel RP, Szer IS, Taylor SI, Taylor-Albert ES, Vehe RK, Vogelgesang SA, Vogler LB, Wallace CA, Wargula JC, White PH, Wilkenfeld MJ, 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. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high frequencies of black race, severe onset, distal weakness, falling episodes, Raynaud phenomenon, cardiac involvement, high CK levels, chronic disease course, frequent hospitalization, and wheelchair use. Characteristic features of the anti-Mi-2 subgroup included Hispanic ethnicity, classic dermatomyositis and malar rashes, high CK levels, and very low mortality. Finally, the most common features of patients without any currently defined MSA or myositis-associated autoantibodies included linear extensor erythema, arthralgia, and a monocyclic disease course. Several demographic and clinical features were shared between juvenile and adult idiopathic inflammatory myopathy subgroups, but with several important differences. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct autoantibody phenotypes defined by varying clinical and demographic characteristics, laboratory features, and outcomes.

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