{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T06:13:27Z","timestamp":1780380807158,"version":"3.54.1"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T00:00:00Z","timestamp":1623456000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T00:00:00Z","timestamp":1623456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Juvenile idiopathic inflammatory myopathies (JIIMs) is a group of autoimmune disorders, including juvenile dermatomyositis (JDM), juvenile polymyositis (JPM) and overlap myositis, that are characterized by proximal muscle weakness, elevated levels of serum muscle enzymes, and pathognomonic skin findings. While the exact etiology of JIIMs is unclear, the presence of myositis specific autoantibodies (MSAs) have been associated with certain clinical phenotypes, organ involvement and disease prognosis. To date, there have been few studies of the associations between MSA presence and patient ethnicity. It is important to understand the extent to which ethnicity impacts disease manifestations, organ involvement and clinical outcomes. The goal of our study is to determine MSA and myositis associated autoantibody (MAA) presence, clinical phenotype, and disease course in a racially diverse population of pediatric patients with JIIMs.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Patients age 2\u201321\u2009years with a prior diagnosis of JDM, JPM or overlap myositis, who had been tested for MSA\/MAA, were eligible for study inclusion. Clinical and laboratory data were collected retrospectively via manual chart review in this single-center study. Descriptive statistics were performed to summarize each variable. Given the small sample size, non-parametric testing was performed using Fischer\u2019s exact test, Wilcoxon rank sum test and Kruskal-Wallis test.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Thirty one patients were included in the analysis. Race and ethnicity were self-reported as Hispanic (48.4%), white (25.8%), and Black (25.8%). The most prevalent MSAs were anti-MDA5 (25.8%), anti-p155\/140 (22.6%) and anti-MJ (19.4%). Presence of autoantibodies (<jats:italic>p<\/jats:italic>\u2009=\u20090.04) and pulmonary disease (<jats:italic>p<\/jats:italic>\u2009=\u20090.03) were significantly higher in patients of Black or Hispanic descent compared with white descent. Anti-MDA5 antibodies, cutaneous ulceration, cardiopulmonary involvement, hospitalizations and one death were only reported in patients with Black or Hispanic descent. Patients with anti-MDA5 antibodies were more likely to be male (p\u2009=\u20090.04) and to have cutaneous ulceration (<jats:italic>p<\/jats:italic>\u2009=\u20090.02).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This study describes the prevalence of MSA\/MAA in a racially diverse group of patients with JIIM and further delineates clinical phenotype and disease complications in these groups. We found a relatively high proportion of children with anti-MDA5 antibodies and described potentially worse clinical courses in children of Black or Hispanic descent. Further investigation is warranted to examine these findings.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12969-021-00574-6","type":"journal-article","created":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T20:02:32Z","timestamp":1623528152000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Myositis autoantibodies in a racially diverse population of children with idiopathic inflammatory myopathies"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4330-9692","authenticated-orcid":false,"given":"Megan Mariko","family":"Perron","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9528-9406","authenticated-orcid":false,"given":"Natalia","family":"Vasquez-Canizares","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3155-2604","authenticated-orcid":false,"given":"Gabriel","family":"Tarshish","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0543-7805","authenticated-orcid":false,"given":"Dawn M.","family":"Wahezi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,6,12]]},"reference":[{"issue":"5","key":"574_CR1","doi-asserted-by":"publisher","first-page":"686","DOI":"10.1136\/annrheumdis-2012-201483","volume":"72","author":"D Lazarevic","year":"2012","unstructured":"Lazarevic D, Pistorio A, Palmisani E, Miettunen P, Ravelli A, Pilkington C, et al. The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis. Ann Rheum Dis. 2012;72(5):686\u201393. https:\/\/doi.org\/10.1136\/annrheumdis-2012-201483.","journal-title":"Ann Rheum Dis"},{"issue":"9631","key":"574_CR2","doi-asserted-by":"publisher","first-page":"2201","DOI":"10.1016\/S0140-6736(08)60955-1","volume":"371","author":"B Feldman","year":"2008","unstructured":"Feldman B, Rider L, Reed A, Pachman L. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet. 2008;371(9631):2201\u201312. https:\/\/doi.org\/10.1016\/S0140-6736(08)60955-1.","journal-title":"Lancet"},{"issue":"3","key":"574_CR3","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1002\/art.11122","volume":"49","author":"EP Mendez","year":"2003","unstructured":"Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A, et al. US incidence of juvenile dermatomyositis, 1995\u20131998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases registry. Arthritis Rheum. 2003;49(3):300\u20135. https:\/\/doi.org\/10.1002\/art.11122.","journal-title":"Arthritis Rheum"},{"issue":"4","key":"574_CR4","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1097\/MD.0b013e31829d08f9","volume":"92","author":"LG Rider","year":"2013","unstructured":"Rider LG, Shah M, Mamyrova G, Huber AM, Rice MM, Targoff IN, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine. 2013;92(4):223\u201343. https:\/\/doi.org\/10.1097\/MD.0b013e31829d08f9.","journal-title":"Medicine"},{"issue":"1","key":"574_CR5","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1111\/joim.12444","volume":"280","author":"LG Rider","year":"2016","unstructured":"Rider LG, Nistala K. The juvenile idiopathic inflammatory myopathies: pathogenesis, clinical and autoantibody phenotypes, and outcomes. J Intern Med. 2016;280(1):24\u201338. https:\/\/doi.org\/10.1111\/joim.12444.","journal-title":"J Intern Med"},{"issue":"4","key":"574_CR6","doi-asserted-by":"publisher","first-page":"R138","DOI":"10.1186\/ar4600","volume":"16","author":"SL Tansley","year":"2014","unstructured":"Tansley SL, Betteridge ZE, Gunawardena H, Jacques TS, Owens CM, Pilkington C, et al. Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study. Arthritis Res Ther. 2014;16(4):R138.","journal-title":"Arthritis Res Ther"},{"key":"574_CR7","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/j.jaut.2017.06.007","volume":"84","author":"SL Tansley","year":"2017","unstructured":"Tansley SL, Simou S, Shaddick G, Betteridge ZE, Almeida B, Gunawardena H, et al. Autoantibodies in juvenile-onset myositis: their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun. 2017;84:55\u201364. https:\/\/doi.org\/10.1016\/j.jaut.2017.06.007.","journal-title":"J Autoimmun"},{"issue":"3","key":"574_CR8","doi-asserted-by":"publisher","first-page":"324","DOI":"10.1093\/rheumatology\/kem359","volume":"47","author":"H Gunawardena","year":"2008","unstructured":"Gunawardena H, Wedderburn LR, North J, Betteridge Z, Dunphy J, Chinoy H, et al. Clinical associations of autoantibodies to a p155\/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology. 2008;47(3):324\u20138. https:\/\/doi.org\/10.1093\/rheumatology\/kem359.","journal-title":"Rheumatology"},{"issue":"4","key":"574_CR9","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1016\/j.jpeds.2010.11.033","volume":"158","author":"I Kobayashi","year":"2011","unstructured":"Kobayashi I, Okura Y, Yamada H. Anti-melanoma differentiation-associated gene 5 antibody is a diagnostic and predictive marker for interstitial lung diseases associated with juvenile dermatomyositis. J Pediatr. 2011;158(4):675\u20137. https:\/\/doi.org\/10.1016\/j.jpeds.2010.11.033.","journal-title":"J Pediatr"},{"issue":"12","key":"574_CR10","doi-asserted-by":"publisher","first-page":"2204","DOI":"10.1093\/rheumatology\/keu259","volume":"53","author":"S Tansley","year":"2014","unstructured":"Tansley S, Betteridge ZE, Shaddick G, Gunawardena H, Arnold K, Wedderburn R, et al. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology. 2014;53(12):2204\u20138. https:\/\/doi.org\/10.1093\/rheumatology\/keu259.","journal-title":"Rheumatology"},{"issue":"9","key":"574_CR11","doi-asserted-by":"publisher","first-page":"1726","DOI":"10.1093\/rheumatology\/keq153","volume":"49","author":"K Hoshino","year":"2010","unstructured":"Hoshino K, Muro Y, Sugiura K, Tomita Y, Nakashima R, Mimori T. Anti-MDA5 and anti-TIF1-gamma antibodies have clinical significance for patients with dermatomyositis. Rheumatology. 2010;49(9):1726\u201333. https:\/\/doi.org\/10.1093\/rheumatology\/keq153.","journal-title":"Rheumatology"},{"issue":"8","key":"574_CR12","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1002\/acr.21992","volume":"65","author":"J Hall","year":"2013","unstructured":"Hall J, Casciola-Rosen L, Samedy L, Werner J, Owoyemi K, Danoff S, et al. Anti-melanoma differentiation- associated protein 5-associated dermatomyositis: expanding the clinical Spectrum. Arthritis Care Res. 2013;65(8):1307\u201315. https:\/\/doi.org\/10.1002\/acr.21992.","journal-title":"Arthritis Care Res"}],"container-title":["Pediatric Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-021-00574-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12969-021-00574-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-021-00574-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T20:03:21Z","timestamp":1623528201000},"score":1,"resource":{"primary":{"URL":"https:\/\/ped-rheum.biomedcentral.com\/articles\/10.1186\/s12969-021-00574-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,12]]},"references-count":12,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["574"],"URL":"https:\/\/doi.org\/10.1186\/s12969-021-00574-6","relation":{},"ISSN":["1546-0096"],"issn-type":[{"value":"1546-0096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,12]]},"assertion":[{"value":"6 February 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 May 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 June 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"A waiver of informed consent and HIPAA authorization were approved by the IRB.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"92"}}