Immune dysregulation as a driver of bronchiolitis obliterans

Bronchiolitis obliterans (BO) is a disease characterized by airway obstruction and fibrosis that can occur in all age groups. Bronchiolitis obliterans syndrome (BOS) is a clinical manifestation of BO in patients who have undergone lung transplantation or hematopoietic stem cell transplantation. Pers...

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Main Authors: Kuimiao Deng, Gen Lu
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-12-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2024.1455009/full
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author Kuimiao Deng
Gen Lu
author_facet Kuimiao Deng
Gen Lu
author_sort Kuimiao Deng
collection DOAJ
description Bronchiolitis obliterans (BO) is a disease characterized by airway obstruction and fibrosis that can occur in all age groups. Bronchiolitis obliterans syndrome (BOS) is a clinical manifestation of BO in patients who have undergone lung transplantation or hematopoietic stem cell transplantation. Persistent inflammation and fibrosis of small airways make the disease irreversible, eventually leading to lung failure. The pathogenesis of BO is not entirely clear, but immune disorders are commonly involved, with various immune cells playing complex roles in different BO subtypes. Accordingly, the US Food and Drug Administration (FDA) has recently approved several new drugs that can alleviate chronic graft-versus-host disease (cGVHD) by regulating the function of immune cells, some of which have efficacy specifically with cGVHD-BOS. In this review, we will discuss the roles of different immune cells in BO/BOS, and introduce the latest drugs targeting various immune cells as the main target. This study emphasizes that immune dysfunction is an important driving factor in its pathophysiology. A better understanding of the role of the immune system in BO will enable the development of targeted immunotherapies to effectively delay or even reverse this condition.
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spelling doaj-art-e51f2010c0364d76b77182a712136df22025-08-20T02:49:29ZengFrontiers Media S.A.Frontiers in Immunology1664-32242024-12-011510.3389/fimmu.2024.14550091455009Immune dysregulation as a driver of bronchiolitis obliteransKuimiao DengGen LuBronchiolitis obliterans (BO) is a disease characterized by airway obstruction and fibrosis that can occur in all age groups. Bronchiolitis obliterans syndrome (BOS) is a clinical manifestation of BO in patients who have undergone lung transplantation or hematopoietic stem cell transplantation. Persistent inflammation and fibrosis of small airways make the disease irreversible, eventually leading to lung failure. The pathogenesis of BO is not entirely clear, but immune disorders are commonly involved, with various immune cells playing complex roles in different BO subtypes. Accordingly, the US Food and Drug Administration (FDA) has recently approved several new drugs that can alleviate chronic graft-versus-host disease (cGVHD) by regulating the function of immune cells, some of which have efficacy specifically with cGVHD-BOS. In this review, we will discuss the roles of different immune cells in BO/BOS, and introduce the latest drugs targeting various immune cells as the main target. This study emphasizes that immune dysfunction is an important driving factor in its pathophysiology. A better understanding of the role of the immune system in BO will enable the development of targeted immunotherapies to effectively delay or even reverse this condition.https://www.frontiersin.org/articles/10.3389/fimmu.2024.1455009/fullbronchiolitis obliteransbronchiolitis obliterans syndromeimmune dysregulationimmune cellsfibrosis
spellingShingle Kuimiao Deng
Gen Lu
Immune dysregulation as a driver of bronchiolitis obliterans
Frontiers in Immunology
bronchiolitis obliterans
bronchiolitis obliterans syndrome
immune dysregulation
immune cells
fibrosis
title Immune dysregulation as a driver of bronchiolitis obliterans
title_full Immune dysregulation as a driver of bronchiolitis obliterans
title_fullStr Immune dysregulation as a driver of bronchiolitis obliterans
title_full_unstemmed Immune dysregulation as a driver of bronchiolitis obliterans
title_short Immune dysregulation as a driver of bronchiolitis obliterans
title_sort immune dysregulation as a driver of bronchiolitis obliterans
topic bronchiolitis obliterans
bronchiolitis obliterans syndrome
immune dysregulation
immune cells
fibrosis
url https://www.frontiersin.org/articles/10.3389/fimmu.2024.1455009/full
work_keys_str_mv AT kuimiaodeng immunedysregulationasadriverofbronchiolitisobliterans
AT genlu immunedysregulationasadriverofbronchiolitisobliterans