{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T05:14:38Z","timestamp":1778130878313,"version":"3.51.4"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T00:00:00Z","timestamp":1778112000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Calcinosis cutis is one of the most refractory complications of dermatomyositis (DM), particularly in patients with anti-nuclear matrix protein 2 (NXP2) autoantibodies. Whether tumor necrosis factor-alpha (TNF-\u03b1) blockade is associated with regression of established calcinosis remains uncertain.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a retrospective, single-center study of nine patients with refractory dermatomyositis or juvenile dermatomyositis treated with TNF-\u03b1 inhibitors (infliximab or adalimumab). Myositis-specific autoantibodies, longitudinal muscle strength (MMT8, CMAS), glucocorticoid exposure, and serial imaging of calcinosis were evaluated. Calcinosis change was analyzed as an exploratory endpoint.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Three patients were anti-NXP2 positive, and six were negative for tested myositis-specific autoantibodies. TNF-\u03b1 blockade was associated with sustained clinical improvement and a marked steroid-sparing effect, with median daily prednisolone dose decreasing from 40\u202fmg at baseline to 5\u202fmg at 12\u202fmonths. Radiographic regression of calcinosis, defined\n                      <jats:italic>a priori<\/jats:italic>\n                      as a \u226520% reduction in maximal lesion diameter on serial imaging, was observed in 2 of 3 anti-NXP2-positive patients after prolonged treatment (12\u201320\u202fmonths): notably, calcinosis was absent at baseline in all anti-NXP2-negative patients, precluding assessment of regression in this subgroup. One patient with pre-existing interstitial lung abnormalities experienced pulmonary deterioration, leading to treatment discontinuation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In this small retrospective cohort, prolonged TNF-\u03b1 blockade was associated with radiographic regression of calcinosis in a subset of anti-NXP2-positive patients, while calcinosis was absent at baseline in antibody-negative patients. These findings generate a hypothesis that antibody-defined subgroups may differ in calcinosis responsiveness and warrant prospective validation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2026.1795210","type":"journal-article","created":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T04:54:35Z","timestamp":1778129675000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Reversibility of calcinosis in anti-NXP2-positive refractory dermatomyositis treated with TNF-\u03b1 blockade: a brief report"],"prefix":"10.3389","volume":"13","author":[{"given":"Hui","family":"Huang","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haiguo","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhidan","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yihong","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huihui","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Na","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Le","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Children\u2019s Hospital of Nanjing Medical University","place":["Nanjing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,7]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1038\/s41572-021-00321-x","article-title":"Idiopathic inflammatory myopathies","volume":"7","author":"Lundberg","year":"2021","journal-title":"Nat Rev Dis Primers"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1038\/nrrheum.2018.33","article-title":"Update on outcome assessment in myositis","volume":"14","author":"Rider","year":"2018","journal-title":"Nat Rev Rheumatol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"1155839","DOI":"10.3389\/fmed.2023.1155839","article-title":"Calcinosis in juvenile dermatomyositis: updates on pathogenesis and treatment","volume":"10","author":"Pinotti","year":"2023","journal-title":"Front Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"102533","DOI":"10.1016\/j.autrev.2020.102533","article-title":"Calcinosis biomarkers in adult and juvenile dermatomyositis","volume":"19","author":"Chung","year":"2020","journal-title":"Autoimmun Rev"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"810785","DOI":"10.3389\/fped.2021.810785","article-title":"Is anti-NXP2 autoantibody a risk factor for calcinosis and poor outcome in juvenile dermatomyositis patients?","volume":"9","author":"Toplak","year":"2022","journal-title":"Front Pediatr"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1007\/s12016-021-08920-y","article-title":"An Italian multicenter study on anti-NXP2 antibodies: clinical and serological associations","volume":"63","author":"Fredi","year":"2022","journal-title":"Clin Rev Allergy Immunol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1016\/j.rdc.2025.07.009","article-title":"Calcinosis in adult and juvenile dermatomyositis","volume":"51","author":"Belina","year":"2025","journal-title":"Rheum Dis Clin N Am"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"101768","DOI":"10.1016\/j.berh.2022.101768","article-title":"Calcinosis in dermatomyositis: origins and possible therapeutic avenues","volume":"36","author":"Davuluri","year":"2022","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"1105432","DOI":"10.3389\/fimmu.2023.1105432","article-title":"Role of macrophages and cytokines in the pathogenesis of calcinosis in inflammatory myopathies","volume":"14","author":"Pipitone","year":"2023","journal-title":"Front Immunol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1186\/s12969-018-0299-9","article-title":"Biologic DMARDs in juvenile dermatomyositis-associated calcinosis: current evidence and challenges","volume":"16","author":"Orandi","year":"2018","journal-title":"Pediatr Rheumatol Online J"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"6234","DOI":"10.3390\/jcm13206234","article-title":"Treatment of calcinosis in dermatomyositis\u2014case report and review of the literature","volume":"13","author":"Fr\u0105czek","year":"2024","journal-title":"J Clin Med"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1007\/s40272-024-00658-2","article-title":"Juvenile dermatomyositis: emerging disease biomarkers and treatment targets","volume":"27","author":"Kim","year":"2025","journal-title":"Paediatr Drugs"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"394","DOI":"10.1002\/pds.3409","article-title":"Association between anti-TNF-\u03b1 therapy and interstitial lung disease","volume":"22","author":"Herrinton","year":"2013","journal-title":"Pharmacoepidemiol Drug Saf"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1795210\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T04:54:46Z","timestamp":1778129686000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1795210\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,7]]},"references-count":13,"alternative-id":["10.3389\/fmed.2026.1795210"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2026.1795210","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,7]]},"article-number":"1795210"}}