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The aim of this study was to identify prognostic factors for poor treatment response to initial GC therapy for AOSD.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Data on newly diagnosed AOSD patients were extracted from our database (<jats:italic>n<\/jats:italic>=71, mean age 51.6 years). The primary outcome was a poor treatment outcome at 4 weeks, which was defined as failure to achieve remission or relapse after achieving remission within 4 weeks, followed by administration of two or more rounds of GC pulse therapy or of any other immunosuppressive drugs.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>The initial mean dose \u00b1 standard deviation of prednisolone was 0.82 \u00b1 0.23 mg\/kg\/day, and 34 (47.3%) patients received GC pulse therapy at week 0. Twenty-nine of 71 patients exhibited a poor treatment outcome at 4 weeks (40.8%). The second round of GC pulse therapy or immunosuppressive drugs was added in 17 or 24 of the 29 patients, respectively. These patients had higher baseline white blood cell (WBC) counts, serum ferritin levels, systemic feature score based on clinical symptoms (modified systemic feature score, mSFS), more hemophagocytic syndrome (HPS) over the 4 weeks, and the higher severity score based on modified Pouchot score or severity index of the Japanese Ministry of Health, Labour and Welfare, than the remaining 42 patients. Multivariable logistic regression model identified baseline WBC count as a prognostic factor for poor outcome (odds ratio per 1000\/\u03bcl increment: 1.12, 95% CI 1.04\u20131.29), while thrombocytopenia, hyperferritinemia, and mSFS at baseline did not achieve statistical significance. Receiver-operating characteristic curve analysis showed that the optimal cut-off for WBC count was 13,050\/\u03bcl. The Kaplan-Meier method showed the cumulative rate of poor treatment outcome to be 60.0% in patients with WBC \u226513,050\/\u03bcl and 23.5% in those with WBC &lt;13,050\/\u03bcl.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>A higher WBC count but not thrombocytopenia, hyperferritinemia, and mSFS at baseline was a significant prognostic factor for poor treatment outcome at week 4 in this retrospective cohort of AOSD patients. Our findings provide important information for determining the initial treatment strategy of newly-diagnosed AOSD.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13075-022-02780-3","type":"journal-article","created":{"date-parts":[[2022,4,29]],"date-time":"2022-04-29T06:04:56Z","timestamp":1651212296000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset Still\u2019s disease"],"prefix":"10.1186","volume":"24","author":[{"given":"Fumiaki","family":"Kondo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7181-7494","authenticated-orcid":false,"given":"Takahiko","family":"Sugihara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natsuka","family":"Umezawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisanori","family":"Hasegawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadashi","family":"Hosoya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoki","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaaki","family":"Mori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinsuke","family":"Yasuda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,4,29]]},"reference":[{"key":"2780_CR1","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1136\/ard.30.2.121","volume":"30","author":"EGL Bywaters","year":"1971","unstructured":"Bywaters EGL. 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Clin Rheumatol. 2021. https:\/\/doi.org\/10.1007\/s10067-021-05722-7 Online ahead of print.","DOI":"10.1007\/s10067-021-05722-7"}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-022-02780-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13075-022-02780-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-022-02780-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,29]],"date-time":"2022-04-29T11:07:32Z","timestamp":1651230452000},"score":1,"resource":{"primary":{"URL":"https:\/\/arthritis-research.biomedcentral.com\/articles\/10.1186\/s13075-022-02780-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,29]]},"references-count":49,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["2780"],"URL":"https:\/\/doi.org\/10.1186\/s13075-022-02780-3","relation":{},"ISSN":["1478-6362"],"issn-type":[{"type":"electronic","value":"1478-6362"}],"subject":[],"published":{"date-parts":[[2022,4,29]]},"assertion":[{"value":"21 October 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 April 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 April 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was conducted in accordance with the Declaration of Helsinki and the Ethical Guidelines for Epidemiological Research in Japan. The study was approved by the Ethics Committee of Tokyo Medical and Dental University (M2019-301).","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":"Tokyo Medical and Dental University received unrestricted research grants for the salary of the members in Department of Lifetime Clinical Immunology from Abbvie Japan; Asahi-Kasei; Ayumi Pharmaceutical Corporation; Chugai Pharmaceutical Co., Ltd.; CSL Behring K.K.; Japan Blood Products Organization; Nippon Kayaku; and UCB Japan Co. Ltd. TS has received research grants from AsahiKASEI Co., Ltd.; Daiichi Sankyo.; and Ono Pharmaceutical. TS has received honoraria from Abbvie Japan Co., Ltd.; AsahiKASEI Co., Ltd.; Astellas Pharma Inc.; Ayumi Pharmaceutical; Bristol Myers Squibb K.K.; Chugai Pharmaceutical Co., Ltd.; Eli Lilly Japan K.K.; Mitsubishi-Tanabe Pharma Co.; Ono Pharmaceutical; Pfizer Japan Inc.; Takeda Pharmaceutical Co. Ltd.; and UCB Japan Co. Ltd. NK has received research grants from Chugai Pharmaceutical Co., Ltd.; UCB Japan Co., Ltd; CSL Behring K.K.; Ayumi Pharmaceutical; Japan Blood Products Organization; and Novartis Pharma K.K. NK has received honoraria from Eisai Co., Ltd.; AbbVie GK; Asahi KASEI Pharma Co.; Novartis Pharma K.K.; Japan Blood Products Organization; Kissei Pharmaceutical Co.LTD.; Ono Pharmaceutical; and Mitsubishi-Tanabe Pharma Co. NK has received medical writing fees from AbbVie GK, Asahi Kasei Pharma Co, and Chugai Pharmaceutical Co., LTD. SY received grants and personal fees from Abbvie, Asahi Kasei, Chugai Pharmaceutical, Ono pharmaceuticals, Eisai, and Tanabe-Mitsubishi Pharmaceutical and personal fees from Eli Lilly, GlaxoSmithKline, and Pfizer. All other authors have declared no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"92"}}