Pulmonary Manifestations of Plasma Cell Type Idiopathic Multicentric Castleman Disease: A Clinicopathological Study in Comparison with IgG4-Related Disease

• Nishimura, Midori Filiz

• Igawa, Takuro

• Gion, Yuka

• Tomita, Sakura

• Inoue, Dai

• Izumozaki, Akira

• Ubara, Yoshifumi

• Nishimura, Yoshito

• Yoshino, Tadashi

• Sato, Yasuharu

抄録

Plasma cell type idiopathic multicentric Castleman disease (PC-iMCD) occasionally manifests as parenchymal lung disease. This study aimed to elucidate the detailed clinicopathological features of lung lesions in PC-iMCD and compare the findings with those in immunoglobulin (Ig) G4-related disease (IgG4-RD), the most difficult differential diagnosis of PC-iMCD. We analyzed the clinicopathological findings and immunohistochemical expression patterns of interleukin-6 (IL-6) and Igs in lung specimens from 16 patients with PC-iMCD and 7 patients with IgG4-RD. Histologically, pulmonary PC-iMCD could not be differentiated from IgG4-RD based on lesion distribution patterns, the number of lymphoid follicles and obliterative vasculitis, or fibrosis types. The eosinophil count was higher in the IgG4-RD group than in the PC-iMCD group (p = 0.004). The IgG4/IgG-positive cell ratio was significantly higher in the IgG4-RD group (p < 0.001). The IgA-positive cell count and IL-6 expression intensity were higher in the PC-iMCD group than in the IgG4-RD group (p < 0.001). Based on these findings, we proposed a new diagnostic approach to differentiate lung lesions of PC-iMCD and IgG4-RD. Our approach can be utilized to stratify patients with suspected lung-dominant PC-iMCD to identify candidates for strong immunosuppressive treatment, including IL-6 blockade, at an early stage.

収録刊行物

• Journal of Personalized Medicine

  Journal of Personalized Medicine 10 (4), 269-, 2020-12-10

  MDPI

キーワード

• plasma cell type idiopathic multicentric Castleman disease
• IL-6
• IgG4-related disease
• immunohistochemistry
• hyper IL-6 syndrome

詳細情報

• CRID: 1050850578859697792
• NII論文ID: 120006952296
• ISSN: 20754426
• Web Site: https://ousar.lib.okayama-u.ac.jp/61268
• 本文言語コード: en
• 資料種別: journal article
• データソース種別:
  • IRDB
  • CiNii Articles