A Systematic Review of the Role of Dysfunctional Wound Healing in the Pathogenesis and Treatment of Idiopathic Pulmonary Fibrosis
| Autor: | Dimitrios Karamichos, Rabab Sharif, Alan Betensley |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty medicine.medical_treatment lcsh:Medicine Disease Review Lung Disorder 03 medical and health sciences Idiopathic pulmonary fibrosis chemistry.chemical_compound 0302 clinical medicine medicine Genetic predisposition extracellular matrix remodeling lung transplantation Lung transplantation Intensive care medicine chronic lung allograft dysfunction business.industry lcsh:R General Medicine Pirfenidone respiratory system medicine.disease idiopathic pulmonary fibrosis 3. Good health respiratory tract diseases 030104 developmental biology 030228 respiratory system Respiratory failure chemistry Immunology Nintedanib business medicine.drug |
| Zdroj: | Journal of Clinical Medicine Journal of Clinical Medicine, Vol 6, Iss 1, p 2 (2016) |
| ISSN: | 2077-0383 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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