{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T11:08:01Z","timestamp":1787224081164,"version":"build-2736575974"},"reference-count":34,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2021,12,21]],"date-time":"2021-12-21T00:00:00Z","timestamp":1640044800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["UL1TR001422"],"award-info":[{"award-number":["UL1TR001422"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"the CureJM Foundation Center of Excellence","award":["R-21 AR077565"],"award-info":[{"award-number":["R-21 AR077565"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Easily accessible biomarkers are urgently needed to evaluate immune activation in Juvenile Dermatomyositis (JDM). The goal of this retrospective study is to define immunological and clinical differences between untreated JDM patients with either normal or elevated (&gt;10 mmol\/L) levels of neopterin, a biomarker of macrophage activation. We included all JDM with neopterin data obtained before initiating medical therapy. We assessed T, B, NK cell populations, muscle enzymes, and disease activity scores for skin (sDAS), muscle (mDAS), total (tDAS), the duration of untreated disease, disease course, and myositis-specific antibody (MSA). Seventy-nine percent of 139 untreated JDM patients had elevated serum neopterin. The group with elevated neopterin had significantly more active disease: tDAS 11.9 vs. 8.1 (p &lt; 0.0001), mDAS 5.8 vs. 3.1 (p &lt; 0.0001), sDAS 6.1 vs. 4.9 (p = 0.0002), aldolase 24.0 vs. 7.6 U\/L (p &lt; 0.0001), von Willebrand factor antigen (p &lt; 0.0001), and ESR 19.8 vs. 11.5 mm\/hr (p = 0.01). The flow cytometry documented both reduced T cells (1494 vs. 2278\/mm3, p = 0.008) and NK cells (145 vs. 240\/mm3, p = 0.003). TNF\u03b1-308AA\/AG polymorphism was more common in children with elevated neopterin than TNF\u03b1-308GG (p 0.05). We conclude that the availability of neopterin data will contribute to the rapid assessment of untreated JDM disease activity.<\/jats:p>","DOI":"10.3390\/diagnostics12010008","type":"journal-article","created":{"date-parts":[[2021,12,22]],"date-time":"2021-12-22T01:08:09Z","timestamp":1640135289000},"page":"8","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Clues to Disease Activity in Juvenile Dermatomyositis: Neopterin and Other Biomarkers"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3357-4863","authenticated-orcid":false,"given":"Amer","family":"Khojah","sequence":"first","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"},{"name":"Division of Allergy & Immunology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"},{"name":"Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4058-7112","authenticated-orcid":false,"given":"Gabrielle","family":"Morgan","sequence":"additional","affiliation":[{"name":"Cure JM Center of Excellence, Stanley Manne Research Center, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8117-574X","authenticated-orcid":false,"given":"Lauren M.","family":"Pachman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"},{"name":"Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"},{"name":"Cure JM Center of Excellence, Stanley Manne Research Center, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,12,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Roberson, E.D.O., Mesa, R.A., Morgan, G.A., Cao, L., Marin, W., and Pachman, L.M. (2021). Transcriptomes of peripheral blood mono-nuclear cells from juvenile dermatomyositis patients show elevated inflammation even when clinically inactive. bioRxiv.","DOI":"10.1101\/2021.05.07.443007"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"727","DOI":"10.5604\/01.3001.0010.3851","article-title":"Neopterin as a marker of cellular immunological response","volume":"71","author":"Michalak","year":"2017","journal-title":"Postepy Hig. Med. 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