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A wealth of evidence suggests the substantial contribution of pro\u2010inflammatory cytokines to the development of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c, but the role of interleukin (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>)\u201018 signaling in this disease still remains elusive. To address this issue, we herein determined serum levels of <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018\u2010binding protein isoform a (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a), a soluble decoy receptor for <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018, by enzyme\u2010linked immunosorbent assay in 57 <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients and 20 healthy controls and evaluated their clinical correlation. Serum <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a levels were higher in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients than in healthy controls, while comparable between diffuse cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c and limited cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients. Although serum <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a levels were not associated with dermal and pulmonary fibrotic parameters in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients, there was a significant positive correlation between serum <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a levels and right ventricular systolic pressure estimated by echocardiography. Furthermore, in 24 <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients who underwent right heart catheterization, serum <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a levels positively correlated with mean pulmonary arterial pressure. As for systemic inflammatory markers, significant positive correlations of circulating <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a levels with erythrocyte sedimentation rate and C\u2010reactive protein were noted. These results suggest that the inhibition of <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018 signaling by <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201018<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>a may be involved in the development of pulmonary vascular involvement leading to pulmonary hypertension and modulate the systemic inflammation in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c.<\/jats:p>","DOI":"10.1111\/1346-8138.13252","type":"journal-article","created":{"date-parts":[[2016,1,18]],"date-time":"2016-01-18T13:01:36Z","timestamp":1453122096000},"page":"912-918","source":"Crossref","is-referenced-by-count":19,"title":["Serum levels of interleukin\u201018\u2010binding protein isoform a: Clinical association with inflammation and pulmonary hypertension in systemic 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Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiro","family":"Inaba","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Graduate School of Medicine The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisataka","family":"Maki","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Graduate School of Medicine The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaru","family":"Hatano","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Graduate School of Medicine The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Yamashita","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine The University of Tokyo Tokyo 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