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Mechanisms of Fibroblast Activation during Fibrotic Tissue Remodeling

Fibrosis. 2024, 2(1), 10002; https://doi.org/10.35534/fibrosis.2024.10002
Aleix Rius Rigau    Clara Dees *   
Department of Internal Medicine 3–Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Universitätsklinikum Erlangen, 91054 Erlangen, Germany
*
Authors to whom correspondence should be addressed.

Received: 31 Dec 2023    Accepted: 22 Feb 2024    Published: 26 Feb 2024   

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Abstract

Fibrosis can occur in almost every organ system. It can occur in single organs, such as in idiopathic pulmonary fibrosis (IPF), or affect multiple organs as in systemic sclerosis (SSc). Fibrotic diseases are recognized as major cause of morbidity and mortality in modern societies due to the dysfunction or loss of function of the affected organs. This dysfunction is caused by progressive deposition of extracellular matrix proteins released by activated fibroblasts. Activation of fibroblasts and differentiation into myofibroblasts is required for physiological tissue remodeling, e.g, during wound healing. Disruption of regulatory mechanisms, however, results in chronic and uncontrolled activity of fibroblasts and myofibroblasts. Intensive research during the past years identified several core pathways of pathophysiological relevance, and described different fibroblast subsets based on their expression profile in fibrotic tissue. Herein, we discuss the molecular changes in fibroblasts leading to persistent activation during fibrotic tissue remodeling with a focus on lung fibrosis and SSc.

Keywords

Fibrosis; Systemic sclerosis; Fibroblast

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