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J. Clin. Med. 2017, 6(1), 2; doi:10.3390/jcm6010002

A Systematic Review of the Role of Dysfunctional Wound Healing in the Pathogenesis and Treatment of Idiopathic Pulmonary Fibrosis

1
Nazih Zuhdi Transplant Institute, Integris Baptist Medical Center, Oklahoma City, OK 73112, USA
2
Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
3
Department of Ophthalmology/Dean McGee Eye Institute, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
*
Author to whom correspondence should be addressed.
Academic Editor: David Barnes
Received: 24 October 2016 / Revised: 6 December 2016 / Accepted: 15 December 2016 / Published: 26 December 2016
(This article belongs to the Special Issue Chronic Respiratory Diseases)
View Full-Text   |   Download PDF [259 KB, uploaded 26 December 2016]   |  

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disorder showcasing an interaction between genetic predisposition and environmental risks. This usually involves the coaction of a mixture of cell types associated with abnormal wound healing, leading to structural distortion and loss of gas exchange function. IPF bears fatal prognosis due to respiratory failure, revealing a median survival of approximately 2 to 3 years. This review showcases the ongoing progress in understanding the complex pathophysiology of IPF and it highlights the latest potential clinical treatments. In IPF, various components of the immune system, particularly clotting cascade and shortened telomeres, are highly involved in disease pathobiology and progression. This review also illustrates two US Food and Drug Administration (FDA)-approved drugs, nintedanib (OFEV, Boehringer Ingelheim, Ingelheim am Rhein, Germany) and pirfenidone (Esbriet, Roche, Basel, Switzerland), that slow IPF progression, but unfortunately neither drug can reverse the course of the disease. Although the mechanisms underlying IPF remain poorly understood, this review unveils the past and current advances that encourage the detection of new IPF pathogenic pathways and the development of effective treatment methods for the near future. View Full-Text
Keywords: idiopathic pulmonary fibrosis; extracellular matrix remodeling; lung transplantation; chronic lung allograft dysfunction idiopathic pulmonary fibrosis; extracellular matrix remodeling; lung transplantation; chronic lung allograft dysfunction
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Betensley, A.; Sharif, R.; Karamichos, D. A Systematic Review of the Role of Dysfunctional Wound Healing in the Pathogenesis and Treatment of Idiopathic Pulmonary Fibrosis. J. Clin. Med. 2017, 6, 2.

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