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Published on: February 15, 2019
Innovative approaches to the therapy of fibrosis
Joao A de Andrade1, Victor J Thannickal
1Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294-0006, USA. joao@uab.edu
Purpose Of Review:
The lung in systemic sclerosis (scleroderma) is susceptible to fibrosis and the ensuing respiratory insufficiency contributes to significant morbidity and mortality in this disease. The lack of effective therapies for pulmonary fibrosis has spurred a re-evaluation of pathobiological paradigms and therapeutic strategies in scleroderma-associated interstitial lung disease and in idiopathic pulmonary fibrosis. The purpose of this review is to examine emerging new therapeutic targets that modulate pro-fibrotic phenotypes of tissue-resident cells and the associated aberrant tissue remodeling responses in fibrotic disorders.
Recent Findings:
Progressive forms of tissue fibrosis, including scleroderma, are characterized by an accumulation of activated mesenchymal cells and their secreted extracellular matrix proteins in association with dysrepair of epithelial and endothelial cells. Recent studies suggest that emergence of cellular phenotypes that perpetuate loss of cellular homeostasis is characteristic of many fibrosis-related clinical syndromes.
Summary:
Therapeutic strategies that modulate the fate/phenotype of reparative structural cells, including epithelial, endothelial, and mesenchymal cells, offer new opportunities for the development of more effective drugs for the treatment of fibrosis.
Insights
New therapeutic targets are emerging for fibrotic disorders like scleroderma-associated interstitial lung disease. Modulating reparative cells offers hope for treating pulmonary fibrosis and improving patient outcomes.
Area of Science:
- Pulmonary Medicine
- Fibrosis Research
- Cellular Biology
Background:
- Systemic sclerosis (scleroderma) frequently involves lung fibrosis, leading to respiratory insufficiency, significant morbidity, and mortality.
- Current therapies for pulmonary fibrosis are limited, necessitating a re-evaluation of disease mechanisms and treatment strategies.
- Scleroderma-associated interstitial lung disease (SSc-ILD) and idiopathic pulmonary fibrosis (IPF) are key areas of focus for new therapeutic development.
Purpose of the Study:
- To review emerging therapeutic targets for fibrotic disorders.
- To examine strategies that modulate pro-fibrotic phenotypes in tissue-resident cells.
- To understand aberrant tissue remodeling responses in fibrotic conditions.
Main Methods:
- Literature review of recent studies on tissue fibrosis.
- Analysis of pathobiological paradigms in scleroderma and idiopathic pulmonary fibrosis.
- Identification of novel therapeutic targets and strategies.
Main Results:
- Progressive fibrosis involves activated mesenchymal cells, excessive extracellular matrix deposition, and dysrepair of epithelial and endothelial cells.
- Cellular phenotypes that disrupt homeostasis are characteristic of many fibrotic syndromes.
- Emerging targets focus on modulating the pro-fibrotic activities of tissue-resident cells.
Conclusions:
- Modulating the fate and phenotype of reparative structural cells (epithelial, endothelial, mesenchymal) presents new therapeutic opportunities.
- Development of more effective drugs for treating fibrosis is a key outcome of targeting these cellular pathways.
- This approach holds promise for managing fibrotic lung diseases like SSc-ILD.
