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CASE REPORT article

Front. Immunol.

Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders

Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1525432

This article is part of the Research Topic Autoimmune Diseases: from molecular mechanisms to therapy development View all articles

Anti-Synthetase Syndrome with Anti-PL-7 Antibody Positive in a Child: A Case Report and Literature Review

Provisionally accepted
  • The Affiliated Hospital of Qingdao University, Qingdao, China

The final, formatted version of the article will be published soon.

    Background: Anti-synthetase syndrome (ASS) is a rare autoimmune myopathy and forms part of the idiopathic inflammatory myopathies (IIMs). A distinctive feature of ASS is the presence of anti-aminoacyl tRNA synthase (ARS) antibodies, which target synthetases, leading to inflammation in muscles, lungs, and other tissues. Case presentation: A case of a 12-year-old Chinese girl with recurrent fever, myalgia, and Gottron's papules is reported. Serum creatine kinase was markedly elevated, and chest CT revealed interstitial changes. Magnetic Resonance Imaging (MRI) of the left thigh indicated soft tissue swelling and interstitial fluid accumulation. Electromyography demonstrated myogenic injury. Pathological examination of a left thigh muscle biopsy revealed local edema, focal lymphocyte infiltration, and proliferation and dilation of interstitial small vessels. The myositis antibody spectrum test was positive for anti-PL-7 antibodies. Treatment with glucocorticoids and methotrexate led to significant improvement in her condition. Conclusion: This case represents the youngest reported patient with PL-7 positive ASS to date.

    Keywords: Anti-synthetase syndrome, Anti-PL-7 antibody, Myositis, case report, ILD

    Received: 09 Nov 2024; Accepted: 17 Feb 2025.

    Copyright: © 2025 LIU, Nie, Zhang, Wang, Lin and Chang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Hong Chang, The Affiliated Hospital of Qingdao University, Qingdao, China

    Disclaimer: 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.

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