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Research ArticlePediatric Rheumatology

Clinical and Serologic Characterization of an Argentine Pediatric Myositis Cohort: Identification of a Novel Autoantibody (anti-MJ) to a 142-kDa Protein

GRACIELA ESPADA, JOSE A. MALDONADO COCCO, NOREEN FERTIG and CHESTER V. ODDIS
The Journal of Rheumatology November 2009, 36 (11) 2547-2551; DOI: https://doi.org/10.3899/jrheum.090461
GRACIELA ESPADA
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JOSE A. MALDONADO COCCO
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NOREEN FERTIG
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CHESTER V. ODDIS
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  • For correspondence: cvo5@pitt.edu
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    Figure 1.

    Seven patients immunoprecipitated proteins of apparent molecular weight 142 kDa from 35S methionine-labeled K562 whole-cell extract, separated on an 8% SDS polyacrylamide 20-cm gel (data not shown). Immunoprecipitation was repeated for these 7 patients and the proteins electrophoretically resolved on a 5% SDS polyacrylamide minigel, enhanced with sodium salicylate and visualized by autoradiography (shown here). Lanes 1–6 represent patients included as “MJ” in Table 2; Lane 7, a patient included as “p155/140” in Table 2. Also shown is a 14C molecular weight marker (Mr).

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    Table 1.

    Demographic features of 64 patients with childhood idiopathic inflammatory myopathies. Of the 17 patients with myositis overlap syndromes, 3 had mixed connective tissue disease, 3 had systemic lupus erythematosus, and the remainder had myositis in overlap with other connective tissue diseases.

    FeatureTotal, n = 64JDM, n = 40JPM, n = 7Myositis Overlap Syndromes, n = 17
    Sex F/M48/1630/105/213/4
    Mean age at onset, yrs8.38.79.810.8
    Mean disease duration, yrs4.543.54.8
    Mean followup, yrs3333.8
    • JDM: Juvenile dermatomyositis; JPM: Juvenile polymyositis.

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    Table 2.

    Clinical features in patients with single myositis-specific autoantibody (MSA) or myositis-associated autoantibody (MAA). Four patients not included in the table had 2 autoantibodies: 2 with PM-Scl/Ku, 1 with PM-Scl/MJ, 1 with MJ/Mi-2. Four patients also possessed the anti-Sm autoantibody, which is not an MSA or MAA.

    Clinical ManifestationMi-2 (n = 4)PM-Scl (n = 2)U1RNP (n = 7)Ku (n=l)MJ (n = 16)p155/140 (n = 14)
    Sex F/M3/11/15/21/012/411/3
    Diagnosis
      JPM————2—
      JDM413—1311
      JOS—14113
    Dysphagia21——53
    Muscular
      Proximal weakness42711414
      Contractures—11—77
      Atrophy41——72
    DM cutaneous signs4—1—1411
    Cutaneous vasculitis—11—68
    Calcinosis1———55
    Interstitial lung disease—12—41
    Arthritis113—69
    Raynaud phenomenon—23—14
    • JOS: juvenile overlap syndrome; JPM: juvenile polymyositis; JDM: juvenile dermatomyositis.

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    Table 3.

    Correlation of disease course with autoantibody in a subset of juvenile myositis patients with 2+ years of followup.

    FeatureMi-2, n = 4PM-Scl, n = 2U1RNP, n = 6Ku, n = 1MJ, n = 10p155/140, n = 13
    Course
      Monocyclic314—510
      Polycyclic112142
    Chronic-continuous————11
    Functional status
      I–II4261713
      III–IV————3—
    Disease activity
      Active111148
      Inactive315—65
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The Journal of Rheumatology
Vol. 36, Issue 11
1 Nov 2009
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Clinical and Serologic Characterization of an Argentine Pediatric Myositis Cohort: Identification of a Novel Autoantibody (anti-MJ) to a 142-kDa Protein
GRACIELA ESPADA, JOSE A. MALDONADO COCCO, NOREEN FERTIG, CHESTER V. ODDIS
The Journal of Rheumatology Nov 2009, 36 (11) 2547-2551; DOI: 10.3899/jrheum.090461

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Clinical and Serologic Characterization of an Argentine Pediatric Myositis Cohort: Identification of a Novel Autoantibody (anti-MJ) to a 142-kDa Protein
GRACIELA ESPADA, JOSE A. MALDONADO COCCO, NOREEN FERTIG, CHESTER V. ODDIS
The Journal of Rheumatology Nov 2009, 36 (11) 2547-2551; DOI: 10.3899/jrheum.090461
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