- Open Access
Journal of Translational Medicine volume 21, Article number: 59 (2023)
We are delighted to announce the launch of Fibrosis a new section of the Journal of Translational Medicine with the purpose of gathering current high-quality research to better understand the process of normal tissue repair as well as the pathogenetic mechanisms responsible for the onset and progression of tissue fibrosis that leads to organ dysfunction and failure. Fibrosis is the common denominator in a variety of chronic diseases including idiopathic pulmonary fibrosis, liver cirrhosis, and ulcerative colitis, among others. These fibrotic diseases affect a vast number of people across the world significantly impacting the quality of their life and increasing health care costs.
Regardless of the organ affected, the dysfunction occurs following the excessive production and deposition of collagen and extracellular matrix by activated myofibroblasts altering the architecture and function of the organ . Oxidative damage, chronic inflammation, and critical signaling cascades have been implicated in the activation of myofibroblasts and the perpetuation of abnormal fibrogenesis [1, 2]. Myofibroblasts are mesenchymal cells with several origins; several experimental studies have described the co-expression of endothelial and mesenchymal cell markers in fibroblasts isolated from animal models of fibrosis suggesting that endothelial cells can differentiate into myofibroblasts during a process called endothelial to mesenchymal transition which is particularly relevant in the pathophysiology of cardiac, renal, and pulmonary fibrosis [3,4,5,6].
The pathogenesis of fibrosis is complex and not fully understood, therefore, the development of more effective therapeutic options has been challenging. There are several cell types and signaling pathways responsible for the development of lung fibrosis following repetitive exposure of the alveolar epithelial cells to a variety of injurious stimuli in combination with individual genetic, epigenetic, and immunological characteristics or predisposition . The use of immunosuppressant drugs is controversial [8, 9]. The oral administration of either of the relatively new antifibrotic agents, pirfenidone and nintedanib, has been shown to safely improve quality of life and slow disease progression [10,11,12].
Liver cirrhosis is an organ-specific fibrosis that compromises important metabolic functions ultimately leading to multisystemic complications and historically death . Liver transplant is still a valuable resource to treat the sickest patients, however, newer antiviral medications have shown promise in restoring liver function and improving life expectancy. These treatment advances in cirrhosis provide encouragement for improved outcomes in other fibrotic diseases.
The goal is early diagnosis and prevention; however, most of these diseases manifest clinically when the affected organ is significantly damaged by fibrotic tissue. The time between initiation of fibrogenesis and symptoms onset varies; during the earliest stages of illness, it is difficult to prognosticate the disease course . The advanced stages of organ-specific fibrosis are usually associated with great deterioration of the overall functional capacity and quality of life with deeply negative implications for the psychosocial health and well-being of patients and caregivers.
Fibrosis has also been further implicated in the proliferation and migration of cancer cells while creating conditions that compromise anti-tumor immunity and treatment response. The collective scientific effort is focused on understanding the interaction of profibrotic molecules and cells with a variety of cancer types aiming to develop anti-fibrotic agents that can also prevent and treat malignancies .
The improved understanding of how fibrosis develops, causes morbidity, and promotes cancer is the foundation for making advances in diagnosis, treatment, and ultimately prevention. Fibrosis will be focused on high-quality research from basic science to clinical trials. The expert members of our Editorial Board are committed to ensuring a productive scientific discussion through the rapid publication of internationally competitive and high-level peer-reviewed articles. We look forward to receiving your thought-provoking contributions to Fibrosis.
Maria ATJ, Bourgier C, Martinaud C, Borie R, Rozier P, Rivière S, Crestani B, Guilpain P. De la fibrogenèse à la fibrose: mécanismes physiopathologiques et présentations cliniques [From fibrogenesis towards fibrosis: pathophysiological mechanisms and clinical presentations]. Rev Med Interne. 2020;41(5):325–9 (French).
Piera-Velazquez S, Mendoza FA, Jimenez SA. Endothelial to Mesenchymal Transition (EndoMT) in the pathogenesis of human fibrotic diseases. J Clin Med. 2016;5(4):45.
Karasek MA. Does transformation of microvascular endothelial cells into myofibroblasts play a key role in the etiology and pathology of fibrotic disease? Med Hypotheses. 2007;68(3):650–5.
Zhou X, Chen X, Cai JJ, Chen LZ, Gong YS, Wang LX, Gao Z, Zhang HQ, Huang WJ, Zhou H. Relaxin inhibits cardiac fibrosis and endothelial-mesenchymal transition via the Notch pathway. Drug Des Devel Ther. 2015;9:4599–611.
He J, Xu Y, Koya D, Kanasaki K. Role of the endothelial-to-mesenchymal transition in renal fibrosis of chronic kidney disease. Clin Exp Nephrol. 2013;17:488–97.
Hashimoto N, Phan SH, Imaizumi K, Matsuo M, Nakashima H, Kawabe T, Shimokata K, Hasegawa Y. Endothelial-mesenchymal transition in bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol. 2010;43:161–72.
Moss BJ, Ryter SW, Rosas IO. Pathogenic mechanisms underlying idiopathic pulmonary fibrosis. Annu Rev Pathol. 2022;24(17):515–46. https://doi.org/10.1146/annurev-pathol-042320-030240 (Epub 2021 Nov 23).
Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Idiopathic pulmonary fibrosis clinical research network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366:1968–77.
Anan K, Kataoka Y, Ichikado K, Kawamura K, Johkoh T, Fujimoto K, Tobino K, Tachikawa R, Ito H, Nakamura T, Kishaba T, Inomata M, Kamitani T, Yamazaki H, Ogawa Y, Yamamoto Y. Early corticosteroid dose tapering in patients with acute exacerbation of idiopathic pulmonary fibrosis. Respir Res. 2022;23(1):291.
Noble PW, Albera C, Bradford WZ, et al. Pirfenidone for idiopathic pulmonary fibrosis: Analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47:243–53.
Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–82.
Glass DS, Grossfeld D, Renna HA, Agarwala P, Spiegler P, DeLeon J, Reiss AB. Idiopathic pulmonary fibrosis: current and future treatment. Clin Respir J. 2022;16(2):84–96. https://doi.org/10.1111/crj.13466 (Epub 2022 Jan 10).
Bernardi M, Angeli P, Claria J, et al. Albumin in decompensated cirrhosis: new concepts and perspectives. Gut. 2020;69(6):1127–38.
Kishaba T. Acute exacerbation of idiopathic pulmonary fibrosis. Medicina (Kaunas). 2019;55(3):70.
Piersma B, Hayward MK, Weaver VM. Fibrosis and cancer: a strained relationship. Biochim Biophys Acta Rev Cancer. 2020;1873(2): 188356.
MS research support from Boehringer ingelheim and Genentech.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Pernia Marin, M., Salvatore, M. Fibrosis. J Transl Med 21, 59 (2023). https://doi.org/10.1186/s12967-022-03789-7