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. 2020 Apr 6;22:69. doi: 10.1186/s13075-020-02160-9

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© The Author(s) 2020

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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Fig. 3 — Proportion of individual genes from 28 normalized IRG-S for JDM highest quartile (JDM-HQ), CANDLE, SAVI. *JDM-HQ gene proportion significantly different from SAVI. ^†JDM-HQ gene proportion significantly different from CANDLE. ^‡JDM-HQ gene proportion significantly different from CANDLE and SAVI. Heat map of median proportion of the respective gene by normalized Z-score with red for highest proportion and dark blue for lowest proportion. Bold indicates values in a diagnosis group(s) significantly higher compared to values in the italicized/underlined group(s). Italicized/underlined indicates values in a diagnosis group(s) significantly lower compared to values in bold group(s). a All 28 IRGs are included. b IFI27 has been removed. Individual gene percentages (%) of the total IRG score, by normalized Z-scores (see the “Methods” section) are shown in alphabetical order by gene as median and interquartile ranges for JDM highest-quartile, CANDLE, and SAVI patients. Medians percentages of each gene by diagnosis may not sum to 100 due to skewing in the group, but they do in individual patients. JDM-HQ (n = 14) included 4 with anti-TIF1 Ab, 4 with anti-MDA4 Ab, 3 with anti-NXP2 Ab, and 3 MSA-neg. The JDM Physician Global Activity (PGA) median is 4.1/10 with an interquartile range of 2.4 to 4.7. Abbreviations: IRG, interferon-regulated gene; JDM-HQ, juvenile dermatomyositis patients with the highest quartile of IRG scores; CANDLE, Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature; SAVI, STING associated Vasculopathy with onset during Infancy; anti-TIF1 Ab, includes subgroup of JDM patients with anti-TIF1 autoantibodies; anti-NXP2 Ab, subgroup of JDM patients with anti-NXP2 autoantibodies; anti-MDA5 Ab, includes subgroup of JDM patients with anti-MDA5 autoantibodies; MSA-neg, includes subgroup of JDM that is negative for myositis-specific autoantibodies; PGA. Physician Global Activity on 0–10 visual analog scale