{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T19:01:20Z","timestamp":1793818880891,"version":"4.6.1"},"reference-count":16,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,10,27]],"date-time":"2017-10-27T00:00:00Z","timestamp":1509062400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30\u2010AR\u2010053503"],"award-info":[{"award-number":["P30\u2010AR\u2010053503"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care & Research"],"published-print":{"date-parts":[[2017,11]]},"abstract":"Objective<\/jats:title>Dermatomyositis (DM<\/jats:styled-content>) patients typically present with proximal weakness and autoantibodies that are associated with distinct clinical phenotypes. We observed that DM<\/jats:styled-content> patients with autoantibodies recognizing the nuclear matrix protein NXP<\/jats:styled-content>\u20102 often presented with especially severe weakness. The aim of this study was to characterize the clinical features associated with anti\u2013NXP<\/jats:styled-content>\u20102 autoantibodies.<\/jats:p><\/jats:sec>Methods<\/jats:title>There were 235 DM<\/jats:styled-content> patients who underwent testing for anti\u2013NXP<\/jats:styled-content>\u20102 autoantibodies. Patient characteristics, including muscle strength, were compared between those with and without these autoantibodies. The number of cancer cases observed in anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive subjects was compared with the number expected in the general population.<\/jats:p><\/jats:sec>Results<\/jats:title>Of the DM<\/jats:styled-content> patients, 56 (23.8%) were anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive. There was no significant difference in the prevalence of proximal extremity weakness in patients with and without anti\u2013NXP<\/jats:styled-content>\u20102. In contrast, anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive patients had more prevalent weakness in the distal arms (35% versus 20%; P<\/jats:italic> = 0.02), distal legs (25% versus 8%; P<\/jats:italic> < 0.001), and neck (48% versus 23%; P<\/jats:italic> < 0.001). Anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive subjects were also more likely to have dysphagia (62% versus 35%; P<\/jats:italic> < 0.001), myalgia (46% versus 25%; P<\/jats:italic> = 0.002), calcinosis (30% versus 17%; P<\/jats:italic> = 0.02), and subcutaneous edema (36% versus 19%; P<\/jats:italic> = 0.01) than anti\u2013NXP<\/jats:styled-content>\u20102\u2010negative patients. Five anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive subjects (9%) had cancer\u2010associated myositis, representing a 3.68\u2010fold increased risk (95% confidence interval 1.2\u20138.6) compared to the expected prevalence in the general population.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>In DM<\/jats:styled-content>, anti\u2013NXP<\/jats:styled-content>\u20102 autoantibodies are associated with subcutaneous edema, calcinosis, and a muscle phenotype characterized by myalgia, proximal and distal weakness, and dysphagia. As anti\u2013NXP<\/jats:styled-content>\u20102\u2010positive patients have an increased risk of cancer, we suggest that they undergo comprehensive cancer screening.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23188","type":"journal-article","created":{"date-parts":[[2017,1,13]],"date-time":"2017-01-13T17:58:10Z","timestamp":1484330290000},"page":"1771-1776","source":"Crossref","is-referenced-by-count":175,"title":["Antinuclear Matrix Protein 2 Autoantibodies and Edema, Muscle Disease, and Malignancy Risk in Dermatomyositis Patients"],"prefix":"10.1002","volume":"69","author":[{"given":"Jemima","family":"Albayda","sequence":"first","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iago","family":"Pinal\u2010Fernandez","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilson","family":"Huang","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cassie","family":"Parks","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Paik","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livia","family":"Casciola\u2010Rosen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonye K.","family":"Danoff","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cheilonda","family":"Johnson","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Christopher\u2010Stine","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Mammen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine, Baltimore, Maryland, and National Institute of Arthritis and Musculoskeletal and Skin Diseases NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,27]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"S139","article-title":"Clinical and serological characterisation of the anti\u2010MJ antibody in childhood myositis [abstract]","volume":"40","author":"Oddis CV","year":"1997","journal-title":"Arthritis Rheum"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2011-200697"},{"key":"e_1_2_6_4_1","first-page":"815","article-title":"Autoantibodies to the P140 autoantigen NXP\u20102 in adult dermatomyositis [abstract]","volume":"60","author":"Betteridge Z","year":"2009","journal-title":"Arthritis Rheum"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar3822"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.24547"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keu259"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2013.10416"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38093"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(91)70153-S"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2004.02.006"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34673"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-210198"},{"key":"e_1_2_6_16_1","doi-asserted-by":"crossref","unstructured":"TiniakouE Pinal\u2010FernandezI LloydTE AlbaydaJ PaikJ WernerJL et\u00a0al.More severe disease and slower recovery in\u00a0younger patients with anti\u20103\u2010hydroxy\u20103\u2010methylglutaryl\u2010coenzyme A reductase\u2010associated autoimmune myopathy. 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