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Published on: February 15, 2019
Fibrosis mechanisms in systemic sclerosis and new potential therapies
Raffaele Barile1, Cinzia Rotondo1, Valeria Rella1
1Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Luigi Pinto 1, 71121, Foggia, Italy.
Abstract:
Systemic sclerosis is a rare rheumatic disease characterized by immune cell activation, tissue fibrosis, and endothelial dysfunction. Extracellular matrix synthesis disorder causes widespread fibrosis, primarily in skin and internal organs. Various factors such as TGFβ, VEGF, Galectin-3, and signaling pathways like Wnt/β-catenin are involved in pathophysiological processes. Treatment lacks a unified approach but combines diverse modalities tailored to disease subtype and progression. Current therapeutic strategies include biologics, JAK inhibitors, and IL-6 pathway modulators. Monoclonal antibodies and hypomethylating agents demonstrate potential in fibrosis inhibition. This review focuses on emerging therapeutic evidence regarding drugs targeting collagen, cytokines, and cell surface molecules in systemic sclerosis, aiming to provide insight into potential innovative treatment strategies.
Insights
Systemic sclerosis involves immune activation and fibrosis. Emerging therapies target collagen, cytokines, and cell surface molecules for innovative treatment strategies.
Area of Science:
- Rheumatology
- Immunology
- Fibrosis Research
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease marked by immune cell activation, endothelial dysfunction, and progressive fibrosis.
- Pathological fibrosis in SSc results from extracellular matrix synthesis disorders, affecting skin and internal organs.
- Key molecular players include TGFβ, VEGF, Galectin-3, and the Wnt/β-catenin pathway.
Purpose of the Study:
- To review emerging therapeutic evidence for systemic sclerosis.
- To focus on innovative treatment strategies targeting key molecular pathways and cellular components.
- To provide insights into potential novel drug targets and therapeutic approaches for SSc.
Main Methods:
- Literature review of current and emerging therapeutic strategies for systemic sclerosis.
- Analysis of drugs targeting collagen, cytokines, and cell surface molecules.
- Examination of biologics, JAK inhibitors, IL-6 pathway modulators, monoclonal antibodies, and hypomethylating agents.
Main Results:
- Current treatments for SSc are diverse and tailored to disease subtype and progression.
- Monoclonal antibodies and hypomethylating agents show promise in inhibiting fibrosis.
- Emerging drugs target specific molecular pathways and cellular interactions involved in SSc pathogenesis.
Conclusions:
- Systemic sclerosis treatment requires a multi-faceted approach.
- Targeting collagen, cytokines, and cell surface molecules represents a promising avenue for innovative SSc therapies.
- Further research into novel therapeutic strategies is crucial for improving patient outcomes in SSc.
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