Pulmonary manifestations of plasma cell type idiopathic multicentric castleman disease: A clinicopathological study in comparison with igg4-related disease

Midori Filiz Nishimura, Takuro Igawa, Yuka Gion, Sakura Tomita, Dai Inoue, Akira Izumozaki, Yoshifumi Ubara, Yoshito Nishimura, Tadashi Yoshino, Yasuharu Sato

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Article number269
Pages (from-to)1-16
Number of pages16
JournalJournal of Personalized Medicine
Volume10
Issue number4
DOIs
Publication statusPublished - Dec 2020

Keywords

  • Hyper IL-6 syndrome
  • IL-6
  • IgG4-related disease
  • Immunohistochemistry
  • Plasma cell type idiopathic multicentric Castleman disease

ASJC Scopus subject areas

  • Medicine (miscellaneous)

Fingerprint

Dive into the research topics of 'Pulmonary manifestations of plasma cell type idiopathic multicentric castleman disease: A clinicopathological study in comparison with igg4-related disease'. Together they form a unique fingerprint.

Cite this