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Inflammatory milieu of muscle biopsies in juvenile dermatomyositis

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Abstract

Juvenile dermatomyositis (JDM) is an inflammatory myopathy which causes severe morbidity and high mortality if untreated. In this study, we aimed to define the T-helper cell profile in the muscle biopsies of JDM patients. Muscle biopsies of twenty-six patients (50% female) were included in the study. Immunohistochemical expression of CD3, CD20, CD138, CD68, IL-17, Foxp3, IFN-ɣ, IFN-alpha and IL-4 was studied and muscle biopsies were scored using the JDM muscle biopsy scoring tool. Inflammatory cells were in small clusters in perimysium and perivascular area or scattered throughout the endomysium in most biopsies; however in 2 biopsies, lymphoid follicle-like big clusters were observed, and in one, there was a very dense and diffuse inflammatory infiltration nearly destroying all the muscle architecture. Seventy-three per cent of the biopsies had T cells, 88% had B cells, 57% had plasma cells, and all had macrophages. As for T-helper cell subtypes, 80% of the biopsies were Th1 positive, 92% Th17 positive and 30% Treg positive. No IL-4 positive inflammatory cell was detected, and only 2 biopsies showed IFN-alpha positivity. The mean JDM biopsy score was 17.6, meaning moderate to severe muscular involvement. Visual analogue score of the pathologist was strongly correlated with histopathological features. B cells, macrophages, plasma cells and T cells constitute the inflammatory milieu of the JDM muscle biopsies. As for T cells, JDM is a disease mainly related with Th1 and Th17 T-helper cell subtypes and to some extend Treg. Th2 cells are not involved in the pathogenesis.

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Acknowledgements

This project was supported by Hacettepe University Scientific Research Projects Coordination Unit with the project number 014 D04 101 012-575.

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Authors and Affiliations

  1. Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey

    Erdal Sag, Yelda Bilginer & Seza Ozen

  2. Pediatric Pathology Unit, Department of Pediatrics, Hacettepe University, Ankara, Turkey

    Gulsev Kale, Zuhal Akcoren, Diclehan Orhan, Safak Gucer & Beril Talim

  3. Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey

    Goknur Haliloglu & Haluk Topaloglu

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  1. Erdal Sag
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  2. Gulsev Kale
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  3. Goknur Haliloglu
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  4. Yelda Bilginer
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Contributions

All authors made substantial contributions to the conception or design of this study, or to the generation, analysis and/or interpretation of data, and agree to be accountable for the integrity of the work herein. All authors reviewed the manuscript and approved the submitted version. No person who fulfils the criteria for authorship has been excluded as an author.

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Correspondence to Erdal Sag.

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This study was approved by Hacettepe University Ethical Committee (GO 13/582-30).

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Sag, E., Kale, G., Haliloglu, G. et al. Inflammatory milieu of muscle biopsies in juvenile dermatomyositis. Rheumatol Int 41, 77–85 (2021). https://doi.org/10.1007/s00296-020-04735-w

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  • DOI: https://doi.org/10.1007/s00296-020-04735-w

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