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Abstract Number: 86

The myositis-specific autoantibody and myositis-associated autoantibody phenotypes in Japanese juvenile idiopathic inflammatory myopathies

Takako Miyamae, Takayuki Kishi and Hisashi Yamanaka, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan

Meeting: 2017 Pediatric Rheumatology Symposium

Keywords: juvenile dermatomyositis, juvenile myositis and myopathy

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Session Information

Date: Thursday, May 18, 2017

Title: Clinical and Therapeutic Poster Session

Session Type: Abstract Submissions

Session Time: 5:30PM-7:00PM

Background/Purpose: Demographics,clinical features,and outcomes among myositis-specific autoantibody (MSA) and myositis-associated autoantibodies (MAAs) subgroups were assessed in children with juvenile idiopathic inflammatory myopathies (JIIM).

Methods: MSAs (anti-TIF-1, anti-MJ, anti-ARS , and anti-MDA-5), and MAAs were evaluated with above clinical information.

Results:  Among 12 JIIM children,MSAs and MAAs were detected in 9 (anti-TIF-1 (n=3), anti-MJ (n=3), anti-MDA-5 (n=2), and anti-ARS (n=1))and 1(anti-Ku). The other two were

negative.Eleven except anti-Ku positive child were consistent with juvenile dermatomyositis.Three patients with anti-TIF-1 mainly had skin

manifestations, 2 of the 3 were diagnosed clinically amyopathic dermatomyositis.Typical skin manifestations and myositis were noted in another 3 patients with anti-MJ.Of the two children with anti-MDA-5, one developed interstitial pneumonia successfully treated intravenous cyclophosphamide pulse therapy,and the other had arthritis intractable to conventional treatments and improved by adalimumab.

Conclusion: The MSAs were highly detected in JIIM patients. Myositis was most severe in anti-MJ positive patients, whereas the skin and extra-skeletal muscles symptoms were mainly noted in patients with anti-TIF-1 and anti-MDA-5.


Disclosure: T. Miyamae, None; T. Kishi, 2; H. Yamanaka, None.

To cite this abstract in AMA style:

Miyamae T, Kishi T, Yamanaka H. The myositis-specific autoantibody and myositis-associated autoantibody phenotypes in Japanese juvenile idiopathic inflammatory myopathies [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 4). https://acrabstracts.org/abstract/the-myositis-specific-autoantibody-and-myositis-associated-autoantibody-phenotypes-in-japanese-juvenile-idiopathic-inflammatory-myopathies/. Accessed .
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