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Nishimura, Midori Filiz Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Igawa, Takuro Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Gion, Yuka Division of Pathophysiology, Okayama University Graduate School of Health Sciences Kaken ID researchmap
Tomita, Sakura Department of Pathology, Tokai University School of Medicine
Inoue, Dai Department of Radiology, Kanazawa University Graduate School of Medical Sciences
Izumozaki, Akira Department of Radiology, Kanazawa University Graduate School of Medical Sciences
Ubara, Yoshifumi Nephrology Center, Toranomon Hospital Kajigaya
Nishimura, Yoshito Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences ORCID publons researchmap
Yoshino, Tadashi Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences Kaken ID publons researchmap
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.
plasma cell type idiopathic multicentric Castleman disease
hyper IL-6 syndrome
Journal of Personalized Medicine
© 2020 by the authors.
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