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Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with s...

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Autores principales: Nishimura, Yoshito, Nishikori, Asami, Sawada, Haruki, Czech, Torrey, Otsuka, Yuki, Nishimura, Midori Filiz, Mizuno, Hiroki, Sawa, Naoki, Momose, Shuji, Ohsawa, Kumiko, Otsuka, Fumio, Sato, Yasuharu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: JSLRT 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9353850/
https://www.ncbi.nlm.nih.gov/pubmed/35249898
http://dx.doi.org/10.3960/jslrt.21038
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author Nishimura, Yoshito
Nishikori, Asami
Sawada, Haruki
Czech, Torrey
Otsuka, Yuki
Nishimura, Midori Filiz
Mizuno, Hiroki
Sawa, Naoki
Momose, Shuji
Ohsawa, Kumiko
Otsuka, Fumio
Sato, Yasuharu
author_facet Nishimura, Yoshito
Nishikori, Asami
Sawada, Haruki
Czech, Torrey
Otsuka, Yuki
Nishimura, Midori Filiz
Mizuno, Hiroki
Sawa, Naoki
Momose, Shuji
Ohsawa, Kumiko
Otsuka, Fumio
Sato, Yasuharu
author_sort Nishimura, Yoshito
collection PubMed
description Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with specific associated autoimmune diseases are unclear. This study retrospectively analyzed the clinicopathological features of iMCD patients focusing on autoantibodies. Among 63 iMCD patients in our database, 19 were positive for at least one autoantibody. Among the 19, we identified five with plasma cell type (PC)-iMCD lymph node histopathology and positive anti-phospholipid antibodies. These patients were likely to have thrombocytopenia, anasarca, fever, reticulin fibrosis or renal insufficiency, organomegaly (TAFRO) symptoms, and thrombotic events. The present study suggests that patients with undiagnosed or atypical autoimmune diseases, including anti-phospholipid syndrome (APS), were treated for iMCD. APS may present with thrombocytopenia or even multi-organ failure, which overlap with clinical presentations of iMCD. Due to differences in the treatment regimen and follow-up, recognition of the undiagnosed autoimmune disease process in those suspected of iMCD is essential. Our study highlights the importance of complete exclusion of differential diagnoses in patients with iMCD in their diagnostic workup.
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spelling pubmed-93538502022-08-15 Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease? Nishimura, Yoshito Nishikori, Asami Sawada, Haruki Czech, Torrey Otsuka, Yuki Nishimura, Midori Filiz Mizuno, Hiroki Sawa, Naoki Momose, Shuji Ohsawa, Kumiko Otsuka, Fumio Sato, Yasuharu J Clin Exp Hematop Original Article Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with specific associated autoimmune diseases are unclear. This study retrospectively analyzed the clinicopathological features of iMCD patients focusing on autoantibodies. Among 63 iMCD patients in our database, 19 were positive for at least one autoantibody. Among the 19, we identified five with plasma cell type (PC)-iMCD lymph node histopathology and positive anti-phospholipid antibodies. These patients were likely to have thrombocytopenia, anasarca, fever, reticulin fibrosis or renal insufficiency, organomegaly (TAFRO) symptoms, and thrombotic events. The present study suggests that patients with undiagnosed or atypical autoimmune diseases, including anti-phospholipid syndrome (APS), were treated for iMCD. APS may present with thrombocytopenia or even multi-organ failure, which overlap with clinical presentations of iMCD. Due to differences in the treatment regimen and follow-up, recognition of the undiagnosed autoimmune disease process in those suspected of iMCD is essential. Our study highlights the importance of complete exclusion of differential diagnoses in patients with iMCD in their diagnostic workup. JSLRT 2022-03-05 /pmc/articles/PMC9353850/ /pubmed/35249898 http://dx.doi.org/10.3960/jslrt.21038 Text en © 2022 by The Japanese Society for Lymphoreticular Tissue Research https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution ShareAlike (CC BY-NC-SA) 4.0 License.
spellingShingle Original Article
Nishimura, Yoshito
Nishikori, Asami
Sawada, Haruki
Czech, Torrey
Otsuka, Yuki
Nishimura, Midori Filiz
Mizuno, Hiroki
Sawa, Naoki
Momose, Shuji
Ohsawa, Kumiko
Otsuka, Fumio
Sato, Yasuharu
Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title_full Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title_fullStr Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title_full_unstemmed Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title_short Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
title_sort idiopathic multicentric castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9353850/
https://www.ncbi.nlm.nih.gov/pubmed/35249898
http://dx.doi.org/10.3960/jslrt.21038
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