How immune cells drive lung scarring in systemic sclerosis
Official title: Pathways regulating profibrotic macrophages in a novel explant model of systemic sclerosis-associated interstitial lung disease
Funded at University of Pittsburgh at Pittsburgh · NIH-11187010 · Data updated 2026-11-12
Learning how immune signals cause lung scarring in people with systemic sclerosis-associated interstitial lung disease using patient lung tissue to point to better treatments.
Quick facts
| Grant type | R21 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Pittsburgh at Pittsburgh NIH-funded |
| Lab location | 1 site (Pittsburgh, United States) |
| Project start | September 6, 2024 |
| Project end | August 31, 2026 |
| Funding year | 2025 |
| Award (this year) | $148,064 |
| NIH project number | 5R21AR084251-02 |
| Project ID | NIH-11187010 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are people with systemic sclerosis who have SSc-associated interstitial lung disease and who can donate lung tissue (for example during transplant or surgical procedures) or receive care at participating centers.
✕Less likely to help
People without SSc-ILD, or those not able or willing to donate lung tissue, are unlikely to directly benefit from participation in this project.
What this research studies
This project uses lung tissue removed during surgery or transplant from people with systemic sclerosis-associated ILD to study the immune cells that drive fibrosis. Scientists will analyze individual cell gene activity with single-cell RNA sequencing and work with a novel lung explant model in the lab. They will focus on macrophages and how cytokines like TGF-β, PDGF, IL-6, and IL-13 change those cells and promote scar-forming myofibroblasts. The team will compare findings to idiopathic pulmonary fibrosis to find pathways that could be targeted by drugs.
Why it matters
Potential benefit: If successful, this work could identify molecular targets to develop or repurpose drugs that slow or prevent lung scarring in SSc-ILD.
How similar studies have gone: Previous single-cell studies have revealed altered cell programs in SSc-ILD and IPF and targeting cytokines like IL-6 has shown benefit in SSc skin, but lung-directed therapeutic targets remain incompletely tested.
Where this research is happening
-
University of Pittsburgh at Pittsburgh
Pittsburgh, United States Active
Researchers
- Principal investigator: Lafyatis, Robert a. — University of Pittsburgh at Pittsburgh
- Study coordinator: Lafyatis, Robert a.
Related research
Other NIH-funded projects on the same topics.
-
Understanding how a cell protein (SLC9A1) drives skin and lung scarring in systemic sclerosis
A five-year NIH grant aims to understand how a specific cell protein called SLC9A1 influences scar-forming cells in systemic sclerosis and related lung disease, with the goal of finding possible therapies.
-
From proteins and genes to new treatments for scar-related lung disease
A large NIH grant is working to map the proteins in the lungs of people with systemic sclerosis to find new targets for drugs that could slow or stop lung fibrosis.
-
Vasculopathy in Systemic Sclerosis and Lung Disease in Adults
A study in adults with systemic sclerosis will look at how blood vessels in the lungs and body relate to the development of lung disease, with the aim of finding new ways to prevent it.
-
Functional markers to guide treatment in systemic sclerosis–related lung disease
A research grant at USC is studying blood biomarkers that show whether medicines are helping people with systemic sclerosis–related interstitial lung disease.
-
Systemic sclerosis-related lung disease: who’s at risk and what blood markers hint at it
A dedicated research grant asks how to identify which people with systemic sclerosis will develop interstitial lung disease and which blood biomarkers can signal that risk.
-
New treatments for lung scarring in systemic sclerosis
This project looks for new drug targets and therapies to help people with systemic sclerosis who have scarring in their lungs.
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page.