Diagnostic criteria, severity classification and guidelines of systemic sclerosis

  • Yoshihide Asano
    Department of Dermatology Faculty of Medicine The University of Tokyo Tokyo Japan
  • Masatoshi Jinnin
    Department of Dermatology and Plastic Surgery Faculty of Life Sciences Kumamoto University Kumamoto Japan
  • Yasushi Kawaguchi
    Institute of Rheumatology Tokyo Woman's Medical University Tokyo Japan
  • Masataka Kuwana
    Department of Allergy and Rheumatology Nippon Medical School Graduate School of Medicine Tokyo Japan
  • Daisuke Goto
    Department of Rheumatology Faculty of Medicine, Univertity of Tsukuba Ibaraki Japan
  • Shinichi Sato
    Department of Dermatology Faculty of Medicine The University of Tokyo Tokyo Japan
  • Kazuhiko Takehara
    Department of Molecular Pathology of Skin Faculty of Medicine Institute of Medical, Pharmaceutical and Health Sciences Kanazawa University Ishikawa Japan
  • Masaru Hatano
    Graduate School of Medicine Department of Therapeutic Strategy for Heart Failure The University of Tokyo Tokyo Japan
  • Manabu Fujimoto
    Department of Dermatology Faculty of Medicine University of Tsukuba Ibaraki Japan
  • Naoki Mugii
    Section of Rehabilitation Kanazawa University Hospital Ishikawa Japan
  • Hironobu Ihn
    Department of Dermatology and Plastic Surgery Faculty of Life Sciences Kumamoto University Kumamoto Japan

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Abstract

<jats:title>Abstract</jats:title><jats:p>Several effective drugs have been identified for the treatment of systemic sclerosis (<jats:styled-content style="fixed-case">SS</jats:styled-content>c). However, in advanced cases, not only their effectiveness is reduced but they may be also harmful due to their side‐effects. Therefore, early diagnosis and early treatment is most important for the treatment of <jats:styled-content style="fixed-case">SS</jats:styled-content>c. We established diagnostic criteria for <jats:styled-content style="fixed-case">SS</jats:styled-content>c in 2003 and early diagnostic criteria for <jats:styled-content style="fixed-case">SS</jats:styled-content>c in 2011, for the purpose of developing evaluation of each organ in <jats:styled-content style="fixed-case">SS</jats:styled-content>c. Moreover, in November 2013, the American College of Rheumatology and the European Rheumatology Association jointly developed new diagnostic criteria for increasing their sensitivity and specificity, so we revised our diagnostic criteria and severity classification of <jats:styled-content style="fixed-case">SS</jats:styled-content>c. Furthermore, we have revised the clinical guideline based on the newest evidence. In particular, the clinical guideline was established by clinical questions based on evidence‐based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of <jats:styled-content style="fixed-case">SS</jats:styled-content>c.</jats:p>

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