Scleroderma Leaders Gather in UK for International Workshop  

Scleroderma researchers from around the world convened at the 19th International Workshop on Scleroderma Research this week at St. John’s College in Cambridge, UK. 

This bi-annual meeting brings together basic, translational and clinical investigators in scleroderma and world leaders in related scientific fields, to promote scientific exchange and provide a venue for advancing the science of scleroderma. 

Several current and past leaders and researchers from the National Scleroderma Foundation shared updates on their research, assisted in the planning of the workshop, and joined to learn and collaborate with others in the field.  

“Interdisciplinary collaboration is crucial in our path to a cure,” said Carol Feghali-Bostwick, PhD, Vice Chair, National Scleroderma Foundation Board of Directors, who also serves on the Workshop’s organizing committee. “Our collaborative community and the open sharing of novel advances in the field positively impact our research progress,” she continued. 

Presentations included groundbreaking progress in cellular therapies and novel pathways, as well as research that has increased our understanding of GI, lung, skin, and cardiac involvement.  A couple of exciting highlights from Foundation-funded projects include:  

  • Dr. Franck Barrat, a National Scleroderma Foundation Established Investigator Award recipient, shared an update on his Foundation supported work looking at altered X-chromosome inactivation of the TLR7/8 locus and its impact on plasmacytoid dendritic cell heterogeneity in systemic sclerosis, particularly in female patients.1  This suggests that the protein locus contributes to ongoing type 1 interferon activity of dendritic cells in women living with scleroderma.  
  • Dr. Michael Whitfield gave an update on his lab’s progress looking at single cell omics of human skin equivalents, including modelling macrophage and fibroblast interactions in systemic sclerosis. This work2 builds on Dr. Patricia Pioli’s Foundation funded work and furthers our understanding of the role of the RUNX1 transcription factor in disease progression in people living with scleroderma.  
  • Dr. Johann Gudjonsson shared new insights into the pathogenesis of skin inflammation. This work was supported in part by the Foundation and looked at skin samples from early disease Systemic Sclerosis patients. Utilizing both single-cell and spatial-seq data, integrated with existing data, this work found specific cells with statistically significant spatially enriched interactions in SSC skin.  
  • Dr. Tracy Frech, Foundation Medical & Scientific Advisory Board Member, moderated a terrific session on the standard of care for systemic sclerosis across the globe and its impact on clinical trial design and conduct. This included updates on clinical guidelines and treatment recommendations3-5 from international standard setting organizations as well as discussion on how practice patterns and standard of care may impact trial design.  

References  

  1. Du, Y., Faz-Lopez, B., Ah Kioon, M. D., Cenac, C., Pierides, M., Lakin, K. S., Spiera, R. F., Chaumeil, J., Truchetet, M. E., Gordon, J. K., Guéry, J. C., & Barrat, F. J. (2025). Altered X-chromosome inactivation of the TLR7/8 locus and heterogeneity of pDCs in systemic sclerosis. The Journal of experimental medicine, 222(3), e20231809. https://doi.org/10.1084/jem.20231809 
  2. RUNX1 is Expressed in a Subpopulation of Dermal Fibroblasts and Higher RUNX1 Levels are Associated with the Severity of Systemic Sclerosis. Rezvan Parvizi, Zhiyun Gong, Helen C. Jarnagin, Diana M. Toledo, Tamar R. Abel, Dillon Popovich, Madeline J. Morrisson, Sasha Shenk, Monique E. Hinchcliff, Jonathan A. Garlick, Patricia A. Pioli, Michael L. Whitfield. bioRxiv 2024.04.03.587966; doi: https://doi.org/10.1101/2024.04.03.587966  
  3. Del Galdo F, Lescoat A, Conaghan PG, et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Annals of the Rheumatic Diseases Published Online First: 11 October 2024. doi: 10.1136/ard-2024-226430   
  4. Denton, et al. The 2024 British Society for Rheumatology guideline for management of systemic sclerosis, Rheumatology, Volume 63, Issue 11, November 2024, Pages 2956–2975, https://doi.org/10.1093/rheumatology/keae394 
  5. Johnson, S. R., et al. (2024). 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis & rheumatology (Hoboken, N.J.), 76(8), 1182–1200. https://doi.org/10.1002/art.42861ATS   
  6. Raghu, G., Montesi, S. B., Silver, R. M., Hossain, T., Macrea, M., Herman, D., Barnes, H., Adegunsoye, A., Azuma, A., Chung, L., Gardner, G. C., Highland, K. B., Hudson, M., Kaner, R. J., Kolb, M., Scholand, M. B., Steen, V., Thomson, C. C., Volkmann, E. R., Wigley, F. M., … Ghazipura, M. (2024). Treatment of Systemic Sclerosis-associated Interstitial Lung Disease: Evidence-based Recommendations. An Official American Thoracic Society Clinical Practice Guideline. American journal of respiratory and critical care medicine, 209(2), 137–152. https://doi.org/10.1164/rccm.202306-1113ST