{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T04:57:12Z","timestamp":1781672232682,"version":"3.54.5"},"reference-count":27,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T00:00:00Z","timestamp":1781654400000},"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>Purpose<\/jats:title>\n                    <jats:p>This study aimed to examine the clinical features and prognosis of patients with distal limb weakness in anti-nuclear matrix protein 2 antibody (anti-NXP2) positive myositis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and methods<\/jats:title>\n                    <jats:p>This retrospective study included the medical records of patients with anti-NXP2 antibody and follow-up data. Clinical features and prognosis of patients with and without distal limb weakness were compared. The differences in groups and survival analysis were analyzed using SPSS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 110 enrolled adult patients with anti-NXP2 positive myositis, 53 showed distal limb weakness. The frequency of distal limb weakness in patients with anti-NXP2 positive myositis was highest when compared with other dermatomyositis (DM) patients. In the patients with anti-NXP2 positive myositis, the mean onset age of distal limb weakness group was younger, when compared with those without distal limb weakness (38.40\u202f\u00b1\u202f13.36 vs. 46.26\u202f\u00b1\u202f15.02\u202fyears,\n                      <jats:italic>p<\/jats:italic>\n                      \u202f=\u202f0.005). They exhibited higher disease activity and experienced higher frequencies of severe muscle weakness, subcutaneous edema, dysphagia, and abnormal ECG (all\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&amp;lt;\u202f0.05). In terms of treatment, 32.1% patients with distal limb weakness received glucocorticoid (GC) pulse, which was higher than those without distal limb weakness (7.0%,\n                      <jats:italic>p<\/jats:italic>\n                      \u202f=\u202f0.001). There were 32 patients reporting relapse. The mean dose of GC (prednisone equivalent) at relapse was higher. Nine patients with distal limb weakness died during follow-up, with infection representing the leading cause of death. The survival rate was not different in patients with and without distal limb weakness.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Adult patients with anti-NXP2 positive myositis and distal limb weakness were younger at disease onset and exhibited increased disease activity, including a higher prevalence of multi-system involvement, as well as a higher incidence of relapse during follow-up.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2026.1873686","type":"journal-article","created":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T04:49:16Z","timestamp":1781671756000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Increased severity and relapse rate in adult anti-nuclear matrix protein 2 antibody positive myositis patients with distal limb weakness: a retrospective study in China"],"prefix":"10.3389","volume":"13","author":[{"given":"Lin","family":"Liang","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanshan","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qilu","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuo","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruilong","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Cardiology, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chao","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianyu","family":"Zhu","sequence":"additional","affiliation":[{"name":"School of Sport and Exercise Rehabilitation, Jinzhou Medical University","place":["Jinzhou, Liaoning, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianghui","family":"Duan","sequence":"additional","affiliation":[{"name":"Department of Radiology, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ling","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Radiology, China-Japan Friendship Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,17]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"2271","DOI":"10.1002\/art.40320","article-title":"2017 European league against rheumatism\/American College of Rheumatology Classification Criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups","volume":"69","author":"Lundberg","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.nmd.2019.10.005","article-title":"239th ENMC international workshop: classification of dermatomyositis, Amsterdam, the Netherlands, 14-16 December 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