Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review

BackgroundAnti-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,...

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Main Authors: Jia Liu, Nana Nie, Ranran Zhang, Dahai Wang, Yi Lin, Hong Chang
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-03-01
Series:Frontiers in Immunology
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Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2025.1525432/full
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author Jia Liu
Nana Nie
Ranran Zhang
Dahai Wang
Yi Lin
Hong Chang
author_facet Jia Liu
Nana Nie
Ranran Zhang
Dahai Wang
Yi Lin
Hong Chang
author_sort Jia Liu
collection DOAJ
description BackgroundAnti-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 presentationA 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.ConclusionThis case represents the youngest reported patient with PL-7 positive ASS to date.
format Article
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institution DOAJ
issn 1664-3224
language English
publishDate 2025-03-01
publisher Frontiers Media S.A.
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series Frontiers in Immunology
spelling doaj-art-f8c9ffe578644c519b00098bc3cd93272025-08-20T02:55:35ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-03-011610.3389/fimmu.2025.15254321525432Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature reviewJia LiuNana NieRanran ZhangDahai WangYi LinHong ChangBackgroundAnti-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 presentationA 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.ConclusionThis case represents the youngest reported patient with PL-7 positive ASS to date.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1525432/fullanti-synthetase syndromeanti-PL-7 antibodymyositiscase reportILD
spellingShingle Jia Liu
Nana Nie
Ranran Zhang
Dahai Wang
Yi Lin
Hong Chang
Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
Frontiers in Immunology
anti-synthetase syndrome
anti-PL-7 antibody
myositis
case report
ILD
title Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
title_full Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
title_fullStr Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
title_full_unstemmed Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
title_short Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review
title_sort anti synthetase syndrome with anti pl 7 antibody positive in a child a case report and literature review
topic anti-synthetase syndrome
anti-PL-7 antibody
myositis
case report
ILD
url https://www.frontiersin.org/articles/10.3389/fimmu.2025.1525432/full
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AT dahaiwang antisynthetasesyndromewithantipl7antibodypositiveinachildacasereportandliteraturereview
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