Fujimoto Shino, Koga Tomohiro, Kawakami Atsushi, Kawabata Hiroshi, Okamoto Shinichiro, Mizuki Masao, Yano Shingo, Ide Makoto, Uno Kazuko, Yagi Katsumi, Kojima Toshiyuki, Mizutani Minoru, Tokumine Yukihiro, Nishimoto Norihiro, Fujiwara Hiroshi, Nakatsuka Shin-Ichi, Shiozawa Kazuko, Iwaki Noriko, Masaki Yasufumi, Yoshizaki Kazuyuki
a Division of Hematology and Immunology, Medicine , Kanazawa Medical University , Ishikawa , Japan.
b Unit of Advanced Preventive Medical Sciences , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan.
Mod Rheumatol. 2018 Jan;28(1):161-167. doi: 10.1080/14397595.2017.1366093. Epub 2017 Sep 7.
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.
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.
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.
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.
确定Castleman病（CD）的暂定诊断标准和疾病严重程度分类，并描述日本人群中人类疱疹病毒8（HHV - 8）阴性的特发性多中心CD（iMCD）的临床和病理特征。
我们在日本成立了CD研究工作组，并召开会议讨论和确定CD的暂定诊断标准和疾病严重程度分类。随后，我们使用日本全国患者登记系统分析了142例被归类为iMCD的患者。
基于讨论，我们提出了CD的初步诊断标准和疾病严重程度分类。此外，我们还提出了疾病活动评分方案。我们确定了根据这些诊断标准诊断出的iMCD患者的临床和病理特征。在疾病严重程度分类中，分别有37%、33%和30%的患者被归类为轻度、中度和重度疾病。
这是日本团队首次提出的CD诊断和分类建议。需要进一步研究以验证它们是否能将CD与其他炎症性疾病区分开来，并确定其敏感性和特异性。
