{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T22:29:35Z","timestamp":1786573775055,"version":"build-2736575974"},"reference-count":33,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T00:00:00Z","timestamp":1780704000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T00:00:00Z","timestamp":1786579200000},"content-version":"vor","delay-in-days":68,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82330097"],"award-info":[{"award-number":["82330097"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Key Research and Development Program of China","award":["2022YFC3602002"],"award-info":[{"award-number":["2022YFC3602002"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"DOI":"10.1186\/s13075-026-03834-6","type":"journal-article","created":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T01:56:57Z","timestamp":1780711017000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Site-level cutaneous phenotyping framework for anti-MDA5-positive dermatomyositis: exploratory associations with systemic immune reference markers"],"prefix":"10.1186","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-5372-136X","authenticated-orcid":false,"given":"Ziyuan","family":"Tian","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-3723-5669","authenticated-orcid":false,"given":"Lingbo","family":"Bi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-9592-638X","authenticated-orcid":false,"given":"Jiahui","family":"Han","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0675-739X","authenticated-orcid":false,"given":"Ke","family":"Xue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6314-3610","authenticated-orcid":false,"given":"Yong","family":"Cui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,6,6]]},"reference":[{"issue":"12","key":"3834_CR1","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1038\/s41584-020-00515-9","volume":"16","author":"Y Allenbach","year":"2020","unstructured":"Allenbach Y, Benveniste O, Stenzel W, Boyer O. Immune-mediated necrotizing myopathy: clinical features and pathogenesis. Nat Rev Rheumatol. 2020;16(12):689\u2013701.","journal-title":"Nat Rev Rheumatol"},{"issue":"9","key":"3834_CR2","doi-asserted-by":"publisher","first-page":"1563","DOI":"10.1093\/rheumatology\/kes102","volume":"51","author":"T Gono","year":"2012","unstructured":"Gono T, Sato S, Kawaguchi Y, et al. Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford). 2012;51(9):1563\u201370.","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"3834_CR3","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1038\/s41584-023-01054-9","volume":"20","author":"X Lu","year":"2024","unstructured":"Lu X, Peng Q, Wang G. Anti-MDA5 antibody-positive dermatomyositis: pathogenesis and clinical progress. Nat Rev Rheumatol. 2024;20(1):48\u201362.","journal-title":"Nat Rev Rheumatol"},{"issue":"12","key":"3834_CR4","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.1111\/resp.70106","volume":"30","author":"C Zong","year":"2025","unstructured":"Zong C, Wu S, Zhu L, et al. The FLATCAN Model: A Novel Score for Predicting Mortality Risk in Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis. Respirology. 2025;30(12):1153\u201364.","journal-title":"Respirology"},{"issue":"4","key":"3834_CR5","doi-asserted-by":"publisher","first-page":"1535","DOI":"10.1016\/j.chest.2020.04.057","volume":"158","author":"X Lian","year":"2020","unstructured":"Lian X, Zou J, Guo Q, et al. Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease: The FLAIR Model. Chest. 2020;158(4):1535\u201345.","journal-title":"Chest"},{"key":"3834_CR6","doi-asserted-by":"publisher","first-page":"e940251","DOI":"10.12659\/MSM.940251","volume":"29","author":"J Guo","year":"2023","unstructured":"Guo J, Mei C, Yu Q, Huang A. Risk Prediction for Rapidly Progressive Interstitial Lung Disease in Anti-MDA5-Positive Dermatomyositis: The CRAFT Model. Med Sci Monit. 2023;29:e940251.","journal-title":"Med Sci Monit"},{"key":"3834_CR7","doi-asserted-by":"publisher","first-page":"1319957","DOI":"10.3389\/fimmu.2023.1319957","volume":"14","author":"H de Boysson","year":"2023","unstructured":"de Boysson H, Cuchet M, Cassius C, et al. Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients. Front Immunol. 2023;14:1319957.","journal-title":"Front Immunol"},{"issue":"12","key":"3834_CR8","doi-asserted-by":"publisher","first-page":"1252","DOI":"10.1001\/jamadermatol.2025.4161","volume":"161","author":"A Srikumar","year":"2025","unstructured":"Srikumar A, Kaltchenko M, Niknejad K, Bao A, McCaffrey T, Kang J. Active Rash, Interstitial Lung Disease, and Neutrophil to Lymphocyte Ratio and Mortality in Dermatomyositis. JAMA dermatology. 2025;161(12):1252\u20137.","journal-title":"JAMA dermatology"},{"key":"3834_CR9","doi-asserted-by":"crossref","unstructured":"Shanshan L, Xin L, Xiaoming S. Calcinosis in adult patients with antimelanoma differentiation-associated gene 5 antibody positive dermatomyositis. J Am Acad Dermatol. 2025;94:1243\u20136.","DOI":"10.1016\/j.jaad.2025.11.092"},{"issue":"4","key":"3834_CR10","doi-asserted-by":"publisher","first-page":"887","DOI":"10.1111\/j.1365-2133.2008.08711.x","volume":"159","author":"RQ Klein","year":"2008","unstructured":"Klein RQ, Bangert CA, Costner M, et al. Comparison of the reliability and validity of outcome instruments for cutaneous dermatomyositis. Br J Dermatol. 2008;159(4):887\u201394.","journal-title":"Br J Dermatol"},{"issue":"3","key":"3834_CR11","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1111\/j.1365-2133.2009.09521.x","volume":"162","author":"M Yassaee","year":"2010","unstructured":"Yassaee M, Fiorentino D, Okawa J, et al. Modification of the cutaneous dermatomyositis disease area and severity index, an outcome instrument. Br J Dermatol. 2010;162(3):669\u201373.","journal-title":"Br J Dermatol"},{"issue":"4","key":"3834_CR12","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1038\/jid.2011.440","volume":"132","author":"R Goreshi","year":"2012","unstructured":"Goreshi R, Okawa J, Rose M, et al. Evaluation of reliability, validity, and responsiveness of the CDASI and the CAT-BM. J Invest Dermatol. 2012;132(4):1117\u201324.","journal-title":"J Invest Dermatol"},{"issue":"1","key":"3834_CR13","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1186\/s12890-023-02706-y","volume":"23","author":"FP Ren","year":"2023","unstructured":"Ren FP, Chen Q, Yao SS, et al. Characteristics and prognostic implications of peripheral blood lymphocyte subsets in patients with anti-MDA5 antibody positive dermatomyositis-interstitial lung disease. BMC Pulm Med. 2023;23(1):411.","journal-title":"BMC Pulm Med"},{"issue":"12","key":"3834_CR14","doi-asserted-by":"publisher","first-page":"1955","DOI":"10.1136\/annrheumdis-2017-211468","volume":"76","author":"IE Lundberg","year":"2017","unstructured":"Lundberg IE, Tj\u00e4rnlund A, Bottai M, et al. 2017 European League Against Rheumatism\/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76(12):1955\u201364.","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"3834_CR15","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.nmd.2019.10.005","volume":"30","author":"AL Mammen","year":"2020","unstructured":"Mammen AL, Allenbach Y, Stenzel W, Benveniste O. 239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14\u201316 December 2018. Neuromuscul. Disord. 2020;30(1):70\u201392.","journal-title":"Neuromuscul. Disord"},{"issue":"1","key":"3834_CR16","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1186\/s12964-025-02159-2","volume":"23","author":"LQ Zhao","year":"2025","unstructured":"Zhao LQ, Yang XQ, Niu Q, et al. MDA5 protein mediating persistent ER stress\/unfolded protein response contributes to endothelial-mesenchymal-transition of lung microvascular endothelial cell in dermatomyositis. Cell communication signaling: CCS. 2025;23(1):149.","journal-title":"Cell communication signaling: CCS"},{"issue":"5","key":"3834_CR17","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1002\/acr.22498","volume":"67","author":"NS Narang","year":"2015","unstructured":"Narang NS, Casciola-Rosen L, Li S, Chung L, Fiorentino DF. Cutaneous ulceration in dermatomyositis: association with anti-melanoma differentiation-associated gene 5 antibodies and interstitial lung disease. Arthritis Care Res. 2015;67(5):667\u201372.","journal-title":"Arthritis Care Res"},{"issue":"3","key":"3834_CR18","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1007\/s12016-015-8496-5","volume":"51","author":"Y Muro","year":"2016","unstructured":"Muro Y, Sugiura K, Akiyama M. Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features-a Comprehensive Review. Clin Rev Allergy Immunol. 2016;51(3):293\u2013302.","journal-title":"Clin Rev Allergy Immunol"},{"issue":"3","key":"3834_CR19","doi-asserted-by":"publisher","first-page":"136","DOI":"10.2478\/rir-2022-0022","volume":"3","author":"F Jia","year":"2022","unstructured":"Jia F, Jiang S, Zhang J, Fu Q, Zhang X, Ye Y. A Preliminary Study of Frequency and Clinical Relevance of Cytotoxic Peripheral CD4 and CD8 T Cells in Patients with Anti-MDA5 Positive Dermatomyositis. Rheumatol Immunol Res. 2022;3(3):136\u201342.","journal-title":"Rheumatol Immunol Res"},{"issue":"1","key":"3834_CR20","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1186\/s13075-025-03639-z","volume":"27","author":"C Weng","year":"2025","unstructured":"Weng C, Liu E, Zheng H, et al. Single-cell sequencing reveals distinct peripheral immune responses in anti-MDA5 antibody positive dermatomyositis with rapidly progressive interstitial lung disease. Arthritis Res Ther. 2025;27(1):189.","journal-title":"Arthritis Res Ther"},{"issue":"2","key":"3834_CR21","first-page":"237","volume":"42","author":"YT Xu","year":"2024","unstructured":"Xu YT, Cao ZY, Lin S, et al. Expansion of exhausted CD8\u2009+\u2009T cells associates with increased pulmonary fungal infection risk in anti-melanoma differentiation associated gene 5 dermatomyositis. Clin Exp Rheumatol. 2024;42(2):237\u201345.","journal-title":"Clin Exp Rheumatol"},{"issue":"4","key":"3834_CR22","doi-asserted-by":"publisher","first-page":"494","DOI":"10.1111\/joim.13607","volume":"293","author":"Q Jin","year":"2023","unstructured":"Jin Q, Fu L, Yang H, et al. Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti-MDA5-positive dermatomyositis. J Intern Med. 2023;293(4):494\u2013507.","journal-title":"J Intern Med"},{"issue":"1","key":"3834_CR23","doi-asserted-by":"publisher","first-page":"6458","DOI":"10.1038\/s41467-022-34145-4","volume":"13","author":"Y Ye","year":"2022","unstructured":"Ye Y, Chen Z, Jiang S, et al. Single-cell profiling reveals distinct adaptive immune hallmarks in MDA5\u2009+\u2009dermatomyositis with therapeutic implications. Nat Commun. 2022;13(1):6458.","journal-title":"Nat Commun"},{"issue":"12","key":"3834_CR24","doi-asserted-by":"publisher","first-page":"1328","DOI":"10.1111\/crj.13720","volume":"17","author":"C Wang","year":"2023","unstructured":"Wang C, Yang H, Li T, et al. Analysis of characteristics related to interstitial lung disease or pulmonary hypertension in patients with dermatomyositis. Clin Respir J. 2023;17(12):1328\u201340.","journal-title":"Clin Respir J"},{"issue":"5","key":"3834_CR25","doi-asserted-by":"publisher","first-page":"786","DOI":"10.1093\/rheumatology\/key386","volume":"58","author":"N Ono","year":"2019","unstructured":"Ono N, Kai K, Maruyama A, et al. The relationship between type 1 IFN and vasculopathy in anti-MDA5 antibody-positive dermatomyositis patients. Rheumatology (Oxford). 2019;58(5):786\u201391.","journal-title":"Rheumatology (Oxford)"},{"issue":"3","key":"3834_CR26","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1111\/bjd.16498","volume":"179","author":"HJ Kim","year":"2018","unstructured":"Kim HJ, Zeidi M, Bonciani D, et al. Itch in dermatomyositis: the role of increased skin interleukin-31. Br J Dermatol. 2018;179(3):669\u201378.","journal-title":"Br J Dermatol"},{"issue":"7","key":"3834_CR27","doi-asserted-by":"publisher","first-page":"2016","DOI":"10.1093\/rheumatology\/kead550","volume":"63","author":"K Yamaguchi","year":"2024","unstructured":"Yamaguchi K, Poland P, Bijoy George T, et al. Correlation between B-cell epitope profile and clinical features of anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford). 2024;63(7):2016\u201323.","journal-title":"Rheumatology (Oxford)"},{"key":"3834_CR28","doi-asserted-by":"publisher","first-page":"773352","DOI":"10.3389\/fimmu.2021.773352","volume":"12","author":"A Nombel","year":"2021","unstructured":"Nombel A, Fabien N, Coutant F. Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies. Front Immunol. 2021;12:773352.","journal-title":"Front Immunol"},{"key":"3834_CR29","doi-asserted-by":"publisher","first-page":"1658547","DOI":"10.3389\/fimmu.2025.1658547","volume":"16","author":"C Gao","year":"2025","unstructured":"Gao C, Wei G, Li C, et al. Combination therapy improves survival prognosis in anti-MDA5-antibody-positive dermatomyositis patients: a single-center retrospective study. Front Immunol. 2025;16:1658547.","journal-title":"Front Immunol"},{"issue":"10","key":"3834_CR30","doi-asserted-by":"publisher","first-page":"1602","DOI":"10.1002\/acr.21728","volume":"64","author":"H Cao","year":"2012","unstructured":"Cao H, Pan M, Kang Y, et al. Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti-melanoma differentiation-associated gene 5 antibody. Arthritis Care Res (Hoboken). 2012;64(10):1602\u201310.","journal-title":"Arthritis Care Res (Hoboken)"},{"issue":"3","key":"3834_CR31","doi-asserted-by":"publisher","first-page":"319","DOI":"10.3899\/jrheum.160682","volume":"44","author":"S Moghadam-Kia","year":"2017","unstructured":"Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Antimelanoma Differentiation-associated Gene 5 Antibody: Expanding the Clinical Spectrum in North American Patients with Dermatomyositis. J Rheumatol. 2017;44(3):319\u201325.","journal-title":"J Rheumatol"},{"issue":"1","key":"3834_CR32","doi-asserted-by":"publisher","first-page":"e70","DOI":"10.1212\/WNL.0000000000009727","volume":"95","author":"Y Allenbach","year":"2020","unstructured":"Allenbach Y, Uzunhan Y, Toquet S, et al. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020;95(1):e70\u20138.","journal-title":"Neurology"},{"issue":"1","key":"3834_CR33","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/j.jaad.2010.09.016","volume":"65","author":"D Fiorentino","year":"2011","unstructured":"Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2011;65(1):25\u201334.","journal-title":"J Am Acad Dermatol"}],"container-title":["Arthritis Research & Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13075-026-03834-6","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-026-03834-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-026-03834-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T22:23:47Z","timestamp":1786573427000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s13075-026-03834-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,6]]},"references-count":33,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["3834"],"URL":"https:\/\/doi.org\/10.1186\/s13075-026-03834-6","relation":{},"ISSN":["1478-6362"],"issn-type":[{"value":"1478-6362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,6]]},"assertion":[{"value":"26 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 May 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 June 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This study was conducted in accordance with the Declaration of Helsinki and approved by the Institutional Ethics Committee of China-Japan Friendship Hospital (approval number: 2023-KY-056). The requirement for informed consent was waived due to the retrospective nature of the study.","order":1,"name":"Ethics","label":"Ethics approval and consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","label":"Consent for publication","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"152"}}