{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T12:58:07Z","timestamp":1790859487732,"version":"4.1.0"},"reference-count":0,"publisher":"The Journal of Rheumatology","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"abstract":"\n Objective<\/jats:title>\n Juvenile dermatomyositis (JDM) is a rare autoimmune disease; when accompanied by interstitial lung disease (ILD), it is a major cause of morbidity and mortality. However, data on clinical features and prognosis of JDM-ILD in Chinese pediatric populations remain limited. The aim of this study was to characterize the clinical features, treatment options, and prognosis of JDM-ILD in Chinese pediatric patients, and to develop a nomogram for predicting clinical response.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n We retrospectively enrolled patients with JDM-ILD from Beijing Children's Hospital (May 2015-January 2026). Demographic, clinical, laboratory, treatment, and prognostic data were collected. Clinical response was assessed through a combination of Pediatric Rheumatology International Trials Organisation (PRINTO)-defined clinically inactive disease (CID), respiratory symptoms, and high-resolution computed tomography (HRCT). Independent prognostic factors were identified using Cox regression analysis and incorporated into a nomogram. Model performance was assessed using concordance index (C-index), calibration curves, and decision curve analysis (DCA).<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n Among the 244 patients included (55.7% female, median onset age 5.7 years), antimelanoma differentiation\u2013associated gene 5 (MDA5; 32%) and antinuclear matrix protein 2 (NXP2; 13.9%) were the most prevalent autoantibodies. At the last follow-up visit, 202 patients (82.8%) achieved CID, with CID rates increasing progressively over time. Three patients who tested positive for anti-MDA5 antibody died of rapidly progressive ILD (RP-ILD; median survival 2.7 months). Multivariable Cox regression identified combination therapy with Janus kinase inhibitors (JAKi; hazard ratio [HR] 2.09, 95% CI 1.25-3.50) or interleukin 6 inhibitors (IL-6i; HR 1.83, 95% CI 1.07-3.12) and Gottron sign (HR 1.80, 95% CI 1.10-2.96) as favorable factors for CID, whereas high-risk status (HR 0.23, 95% CI 0.10-0.57), higher Disease Activity Score (DAS) skin score (HR 0.71, 95% CI 0.48-1.00), and higher neutrophil-to-lymphocyte ratio (NLR; HR 0.84, 95% CI 0.72-0.97) predicted lower probability of CID. The nomogram showed predictive performance in both the training cohort (area under the curve [AUC] 0.67, 0.77, and 0.78 at 1, 3, and 5 years, respectively) and the testing cohort (AUC 0.60, 0.78, and 0.77 at 1, 3, and 5 years, respectively).<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n This nomogram for predicting clinical response in Chinese pediatric patients with JDM-ILD integrates clinical features and treatment variables to enable individualized risk stratification. Combination therapy with JAKi or IL-6i demonstrates promising efficacy and may improve outcomes.<\/jats:p>\n <\/jats:sec>","DOI":"10.3899\/jrheum.2026-0380","type":"journal-article","created":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T12:20:16Z","timestamp":1789474816000},"page":"jrheum.2026-0380","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Features and a Nomogram for Predicting Clinical Response in Juvenile Dermatomyositis\u2013Associated Interstitial Lung Disease"],"prefix":"10.3899","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6055-2697","authenticated-orcid":false,"given":"Xinwei","family":"Shi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1636-9382","authenticated-orcid":false,"given":"Junmei","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2549-7493","authenticated-orcid":false,"given":"Shipeng","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-1067-4076","authenticated-orcid":false,"given":"Xiaohua","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3292-4676","authenticated-orcid":false,"given":"Jianghong","family":"Deng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiying","family":"Kuang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-8217-6765","authenticated-orcid":false,"given":"Caifeng","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2026,9,15]]},"container-title":["The Journal of Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3899\/jrheum.2026-0380","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T12:15:21Z","timestamp":1790856921000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.jrheum.org\/lookup\/doi\/10.3899\/jrheum.2026-0380"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,15]]},"references-count":0,"alternative-id":["10.3899\/jrheum.2026-0380"],"URL":"https:\/\/doi.org\/10.3899\/jrheum.2026-0380","relation":{},"ISSN":["0315-162X","1499-2752","0315-162X"],"issn-type":[{"value":"0315-162X","type":"print"},{"value":"1499-2752","type":"electronic"},{"value":"0315-162X","type":"print"}],"subject":[],"published":{"date-parts":[[2026,9,15]]}}}