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This study aimed to evaluate factors associated with refractory ANM-SRP.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Clinical and pathological data from 48 patients with ANM-SRP were collected. We followed up clinical symptoms and image changes over 12\u2009months. Univariate and multivariate analyses were undertaken to determine the associations between variables of interest and poor response to therapy. Refractory ANM-SRP appeared in 32.5% of patients who showed no or minimal improvement after 12\u2009months of steroid therapy. The clinical risk factors for refractory patients were being male (OR, 19.57;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001), severe muscle weakness (OR, 7.51;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001) and concurrent interstitial lung disease (OR, 39.70;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001). The imaging refractory-related factor was the fatty infiltration rate of thigh muscles over 3\u2009months (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.022) and the pathological factor associated with refractory ANM-SRP was the high expression of B cell activating factor receptor (BAFF-R) in muscle (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.036).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Being male, severe muscle weakness, concurrent interstitial lung disease, quick development of muscle fatty infiltration and more BAFF-R and B lymphocyte infiltration in muscle indicate a poor response to immunosuppressive therapy in patients with ANM-SRP.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-020-01431-7","type":"journal-article","created":{"date-parts":[[2020,7,8]],"date-time":"2020-07-08T07:03:54Z","timestamp":1594191834000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Factors associated with refractory autoimmune necrotizing myopathy with anti-signal recognition particle antibodies"],"prefix":"10.1186","volume":"15","author":[{"given":"Yawen","family":"Zhao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yilin","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaoxia","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3282-9123","authenticated-orcid":false,"given":"Yun","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,8]]},"reference":[{"key":"1431_CR1","doi-asserted-by":"publisher","first-page":"420","DOI":"10.1136\/jnnp.73.4.420","volume":"73","author":"T Miller","year":"2002","unstructured":"Miller T, AI-Lozi MT, Lopate G, Pestronk A. 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Informed consent for all examinations was obtained from the patients or their guardians. The protocol in the study strictly followed the rules of the Declaration of Helsinki.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no conflicts of interest.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"181"}}