Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan

Shino Fujimoto, Tomohiro Koga, Atsushi Kawakami, Hiroshi Kawabata, Shinichiro Okamoto, Masao Mizuki, Shingo Yano, Makoto Ide, Kazuko Uno, Katsumi Yagi, Toshiyuki Kojima, Minoru Mizutani, Yukihiro Tokumine, Norihiro Nishimoto, Hiroshi Fujiwara, Shin ichi Nakatsuka, Kazuko Shiozawa, Noriko Iwaki, Yasufumi Masaki, Kazuyuki Yoshizaki

Research output: Contribution to journalArticlepeer-review

25 Citations (Scopus)

Abstract

Objectives: To determine the tentative diagnostic criteria and disease severity classification for Castleman disease (CD) and describe the clinical and pathologic features among human herpesvirus 8 (HHV-8) negative idiopathic multicentric CD (iMCD) in the Japanese population. Methods: We established the working groups for the research of CD in Japan and had meetings to discuss and define the tentative diagnostic criteria and disease severity classification for CD. We subsequently analyzed 142 patients classified into iMCD by using the nationwide Japanese patient registry. Results: We proposed the preliminary diagnostic criteria and disease severity classification for CD based on our discussion. In addition, we made a proposal for the disease activity score. We identified clinical and pathological features of patients with iMCD diagnosed by these diagnostic criteria. In the disease severity classification, 37, 33 and 30% patients were categorized into mild, moderate and severe diseases, respectively. Conclusion: This is the first proposal for diagnosis and classification of CD by the Japanese group. Further studies are required to validate whether they can distinguish CD from other inflammatory diseases and to determine their sensitivity and specificity.

Original languageEnglish
Pages (from-to)161-167
Number of pages7
JournalModern rheumatology
Volume28
Issue number1
DOIs
Publication statusPublished - 2018 Jan 2

Keywords

  • Castleman disease
  • TAFRO syndrome
  • diagnostic criteria
  • disease severity classification
  • idiopathic multicentric Castleman disease

ASJC Scopus subject areas

  • Medicine(all)

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