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

Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan.
Author Information (click to view)

Fujimoto S, Koga T, Kawakami A, Kawabata H, Okamoto S, Mizuki M, Yano S, Ide M, Uno K, Yagi K, Kojima T, Mizutani M, Tokumine Y, Nishimoto N, Fujiwara H, Nakatsuka SI, Shiozawa K, Iwaki N, Masaki Y, Yoshizaki K,


Fujimoto S, Koga T, Kawakami A, Kawabata H, Okamoto S, Mizuki M, Yano S, Ide M, Uno K, Yagi K, Kojima T, Mizutani M, Tokumine Y, Nishimoto N, Fujiwara H, Nakatsuka SI, Shiozawa K, Iwaki N, Masaki Y, Yoshizaki K, (click to view)

Fujimoto S, Koga T, Kawakami A, Kawabata H, Okamoto S, Mizuki M, Yano S, Ide M, Uno K, Yagi K, Kojima T, Mizutani M, Tokumine Y, Nishimoto N, Fujiwara H, Nakatsuka SI, Shiozawa K, Iwaki N, Masaki Y, Yoshizaki K,

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Modern rheumatology 2017 09 07() 1-7 doi 10.1080/14397595.2017.1366093

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.

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