{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T02:04:11Z","timestamp":1793930651119,"version":"4.8.0"},"reference-count":34,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2018,10,5]],"date-time":"2018-10-05T00:00:00Z","timestamp":1538697600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>There have been no established parameters to predict responsiveness to i.v. cyclophosphamide (<jats:styled-content style=\"fixed-case\">IVCY<\/jats:styled-content>) pulse therapy in combination with corticosteroids in patients with interstitial lung disease (<jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>) related to systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c). This retrospective study was conducted to determine predictive factors for efficacy of <jats:styled-content style=\"fixed-case\">IVCY<\/jats:styled-content> at the time of before and during the treatment. Thirty\u2010two Japanese <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients, ever treated for <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content> with <jats:styled-content style=\"fixed-case\">IVCY<\/jats:styled-content> in combination with prednisolone, were analyzed retrospectively. We performed detailed time\u2010course analyses of parameters derived from blood samples and pulmonary function tests. With the exclusion of eight unclassified patients, 24 patients were classified into 14 good responders (<jats:styled-content style=\"fixed-case\">GR<\/jats:styled-content>) or 10 poor responders (<jats:styled-content style=\"fixed-case\">PR<\/jats:styled-content>) on the basis of changes in percent predicted diffusing capacity for carbon monoxide (<jats:styled-content style=\"fixed-case\">DL<\/jats:styled-content>co). Pretreatment percent predicted <jats:styled-content style=\"fixed-case\">DL<\/jats:styled-content>co was significantly reduced in <jats:styled-content style=\"fixed-case\">PR<\/jats:styled-content> compared with <jats:styled-content style=\"fixed-case\">GR<\/jats:styled-content>. In addition, serum parameters such as Krebs von den Lungen\u20106 (<jats:styled-content style=\"fixed-case\">KL<\/jats:styled-content>\u20106), surfactant protein D (<jats:styled-content style=\"fixed-case\">SP<\/jats:styled-content>\u2010D) and C\u2010reactive protein were significantly higher in <jats:styled-content style=\"fixed-case\">PR<\/jats:styled-content> than in <jats:styled-content style=\"fixed-case\">GR<\/jats:styled-content>. Furthermore, our time\u2010course analyses revealed a transient increase in serum <jats:styled-content style=\"fixed-case\">KL<\/jats:styled-content>\u20106 levels with a peak at 3 months after the first infusion of cyclophosphamide, which showed no relation to therapeutic efficacy. Moreover, continuously high serum <jats:styled-content style=\"fixed-case\">KL<\/jats:styled-content>\u20106 levels (&gt;2000 U\/mL) and rapid decrease in <jats:styled-content style=\"fixed-case\">SP<\/jats:styled-content>\u2010D levels (&lt;200 ng\/mL) during <jats:styled-content style=\"fixed-case\">IVCY<\/jats:styled-content> were remarkably characteristic of <jats:styled-content style=\"fixed-case\">PR<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">GR<\/jats:styled-content>, respectively. <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content> severity\/activity before treatment and variability of serum <jats:styled-content style=\"fixed-case\">KL<\/jats:styled-content>\u20106 and <jats:styled-content style=\"fixed-case\">SP<\/jats:styled-content>\u2010D levels during treatment may be useful to predict therapeutic effects of <jats:styled-content style=\"fixed-case\">IVCY<\/jats:styled-content> on <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010ILD.<\/jats:p>","DOI":"10.1111\/1346-8138.14669","type":"journal-article","created":{"date-parts":[[2018,10,6]],"date-time":"2018-10-06T09:39:41Z","timestamp":1538818781000},"page":"1425-1433","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Prediction of therapeutic response before and during i.v. cyclophosphamide pulse therapy for interstitial lung disease in systemic sclerosis: A longitudinal observational study"],"prefix":"10.1111","volume":"45","author":[{"given":"Hayakazu","family":"Sumida","sequence":"first","affiliation":[{"name":"Department of Dermatology Faculty of Medicine The University of Tokyo Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5560-9778","authenticated-orcid":false,"given":"Yoshihide","family":"Asano","sequence":"additional","affiliation":[{"name":"Department of Dermatology 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