Journal Article Review Ppublish
PubMed: 40817801 DOI: 10.1177/17534666251366023
The fibrosis puzzle of systemic sclerosis-associated ILD and the quest for targeted interventions.
Ther Adv Respir Dis 19:17534666251366023
Authors: Bohdana Doskaliuk, Liubomyr Zaiats, Nazar Sahan, Yuliya Fedorchenko, Olga Antymys, Roman Yatsyshyn
Affiliations
- Department of Pathophysiology, Ivano-Frankivsk National Medical University, Halytska str. 2, Ivano-Frankivsk 76018, Ukraine.
- Department of Pathophysiology, Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine.
- Department of Human Anatomy, Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine.
- Academician Ye. M. Neiko Department of Internal Medicine #1, Clinical Immunology and Allergology, Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine.
Abstract
Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a leading cause of morbidity and mortality in systemic sclerosis, marked by complex immunopathogenic mechanisms and progressive fibrosis. Recent advances have illuminated key cellular players as T cells, macrophages, dendritic cells, and profibrotic mediators such as TGF-β, PDGF, and CTGF, alongside emerging roles for epigenetic reprogramming in sustaining fibroblast activation. This narrative review synthesizes current understanding of immune-fibrotic crosstalk and critically evaluates both established therapies (e.g., mycophenolate mofetil, nintedanib, tocilizumab, and rituximab) and novel targeted approaches. Particular attention is given to emerging immunomodulatory, antifibrotic, and cell-based therapies, including CAR-T and mesenchymal stem cell treatments, as well as the potential of epigenetic modulators repurposed from oncology. By bridging basic science and clinical practice, this review outlines the evolving therapeutic landscape of SSc-ILD and highlights opportunities for future research and personalized intervention.
MeSH
Cells, Sclerosis, Microscopy, Electron, Scanning Transmission, Fibrosis, Therapeutics, Scleroderma, Systemic, Mycophenolic Acid, Lung Diseases, Interstitial