The Role of Infection and Abnormal Immune Responses in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

The immune system is receiving increasing attention for interstitial lung diseases, as knowledge on its role in fibrosis development and response to therapies is expanding. Uncontrolled immune responses and unbalanced injury-inflammation-repair processes drive the initiation and progression of idiopathic pulmonary fibrosis. The regulatory immune system plays important roles in controlling pathogenic immune responses, regulating inflammation and modulating the transition of inflammation to fibrosis. This review aims to summarize and critically discuss the current knowledge on the potential role of regulatory immune cells, including mesenchymal stromal/stem cells, regulatory T cells, regulatory B cells, macrophages, dendritic cells and myeloid-derived suppressor cells in idiopathic pulmonary fibrosis. Furthermore, we review the emerging role of regulatory immune cells in anti-fibrotic therapy and lung transplantation. A comprehensive understanding of immune regulation could pave the way towards new therapeutic or preventive approaches in idiopathic pulmonary fibrosis.

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ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS: THE POSSIBLE ROLE OF BACTERIAL INFECTION AND THE PATTERN OF ITS ANTIBIOTIC RESISTANCE IN PATIENTS ADMITTED TO HOSPITAL

CHEST Journal ◽

10.1378/chest.136.4_meetingabstracts.116s-c ◽

2009 ◽

Vol 136 (4) ◽

pp. 116S

Author(s):

Mohamed M. Metwally ◽

Heba G. Rashed ◽

Mohamed Z. Abokrisha

Keyword(s):

Antibiotic Resistance ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Bacterial Infection ◽

Acute Exacerbation

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The role of infection in acute exacerbation of Idiopathic Pulmonary Fibrosis

10.1183/13993003.congress-2018.pa2913 ◽

2018 ◽

Author(s):

Dong Weng ◽

Huiping Li

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation

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Therapeutic Role of Recombinant Human Soluble Thrombomodulin for Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Medicina ◽

10.3390/medicina55050172 ◽

2019 ◽

Vol 55 (5) ◽

pp. 172 ◽

Cited By ~ 2

Author(s):

Takuma Isshiki ◽

Susumu Sakamoto ◽

Sakae Homma

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Vascular Endothelial Cells ◽

High Mobility ◽

Vascular Endothelial ◽

Therapeutic Role ◽

Soluble Thrombomodulin ◽

Recombinant Human Soluble Thrombomodulin

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an acute respiratory worsening of unidentifiable cause that sometimes develops during the clinical course of IPF. Although the incidence of AE-IPF is not high, prognosis is poor. The pathogenesis of AE-IPF is not well understood; however, evidence suggests that coagulation abnormalities and inflammation are involved. Thrombomodulin is a transmembranous glycoprotein found on the cell surface of vascular endothelial cells. Thrombomodulin combines with thrombin, regulates coagulation/fibrinolysis balance, and has a pivotal role in suppressing excess inflammation through its inhibition of high-mobility group box 1 protein and the complement system. Thus, thrombomodulin might be effective in the treatment of AE-IPF, and we and other groups found that recombinant human soluble thrombomodulin improved survival in patients with AE-IPF. This review summarizes the existing evidence and considers the therapeutic role of thrombomodulin in AE-IPF.

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Acute exacerbation of idiopathic pulmonary fibrosis: Role of Chlamydophila pneumoniae infection

Respirology ◽

10.1111/j.1440-1843.2007.01119.x ◽

2007 ◽

Vol 12 (5) ◽

pp. 700-706 ◽

Cited By ~ 23

Author(s):

Hiromi TOMIOKA ◽

Toshiyasu SAKURAI ◽

Kimio HASHIMOTO ◽

Hironobu IWASAKI

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Chlamydophila Pneumoniae

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The Role of Infection in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a3357 ◽

2019 ◽

Author(s):

D. Weng ◽

H. Li

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation

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Essential role of IL-23 in the development of acute exacerbation of pulmonary fibrosis

AJP Lung Cellular and Molecular Physiology ◽

10.1152/ajplung.00582.2020 ◽

2021 ◽

Author(s):

Satoru Senoo ◽

Akihiko Taniguchi ◽

Junko Itano ◽

Naohiro Oda ◽

Daisuke Morichika ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Bronchoalveolar Lavage Fluid ◽

Lavage Fluid ◽

Neutrophilic Inflammation ◽

Interleukin 17A ◽

Cytokine Levels ◽

Interleukin 23

Acute exacerbation of idiopathic pulmonary fibrosis has a poor prognosis associated with neutrophilic inflammation. Interleukin-23 is a proinflammatory cytokine involved in neutrophilic inflammation. However, little is known about its role in acute exacerbation of pulmonary fibrosis. This study was performed to determine the role of interleukin-23 in acute exacerbation of pulmonary fibrosis. For assessment of acute exacerbation of pulmonary fibrosis, mice were intratracheally administered bleomycin followed by lipopolysaccharide. Inflammatory cells, cytokine levels, and morphological morphometry of the lungs were analyzed. Cytokine levels were measured in the bronchoalveolar lavage fluid of idiopathic pulmonary fibrosis patients with or without acute exacerbation. Interleukin-23, -17A, and -22 levels were increased in the airway of mice with acute exacerbation of pulmonary fibrosis. Interleukin-23p19-deficient mice with acute exacerbation of pulmonary fibrosis had markedly reduced airway inflammation and fibrosis associated with decreased levels of interleukin-17A and -22 compared with wild-type mice. Treatment with an anti-interleukin-23 antibody attenuated airway inflammation and fibrosis and reduced interleukin-17A and -22 levels in mice with acute exacerbation of pulmonary fibrosis. T helper 17 cells were the predominant source of interleukin-17A in mice with acute exacerbation of pulmonary fibrosis. Interleukin-23 levels in bronchoalveolar lavage fluid tended to be higher in idiopathic pulmonary fibrosis patients with than without acute exacerbation. The data presented here suggest that interleukin-23 is essential for the development of acute exacerbation of pulmonary fibrosis, and that blockade of interleukin-23 may be a new therapeutic strategy for acute exacerbation of pulmonary fibrosis.

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