Vascular Working Group

Summary of Current Projects:

• Ongoing validation of ASRAP questionnaire for assessing SSc-RP
• Development and validation of novel PRO instrument for assessing SSc-DU (SSiDU questionnaire)
• Develop and validate new tools for assessing telangiectasia in SSc

Progress to date for working group:

• Published manuscripts outlining the development and validation of ASRAP questionnaire (PIs Pauling & Domsic)
• Awarded the 2021 Betty Benedict Award to assess responsiveness of infrared thermography and ASRAP following PDEVi use on digital vascular compromise in SSc (SATISS study, PIs Murray and Herrick)
• Been awarded 2024 Betty Benedict Award to further develop and validate the SSiDU questionnaire by conducting an international multicentre validation study (100 DU study) PIs Hughes & Pauling

Next steps:

• To complete international multicentre 100 DU Study 2025-2027
• Undertaking additional analysis of ASRAP dataset to develop new tools for assessing telangiectasia in SSc
• Publish findings from SATISS study

What are expected deliverables and timelines for grant/working group?

• Recruitment of SSc patients to SSiDU 100 DU study (2025-2027)
• Draft manuscripts with additional analyses from ASRAP validation study on telangiectasia in SSc
• Draft manuscript reporting outcome of SATISS study

Publications:

1. Pauling JD, Yu L, Frech TM, Herrick AL, Hummers LK, Shah AA, Denton CP, Saketkoo LA, Withey J, Khanna D, Domsic RT. Construct validity and reliability of the Assessment of Systemic Sclerosis-Associated Raynaud’s Phenomenon (ASRAP) questionnaire. Rheumatology (Oxford). 2024 May 2;63(5):1281-1290.
   
   
2. Yu L, Domsic RT, Saketkoo LA, Withey J, Frech TM, Herrick AL, Hummers LK, Shah AA, Denton CP, Khanna D, Pauling JD. Assessment of the Systemic Sclerosis-Associated Raynaud’s Phenomenon Questionnaire: Item Bank and Short-Form Development. Arthritis Care Res (Hoboken). 2023 Aug;75(8):1725-1734.
   
   
3. Patient experiences of digital ulcer development and evolution in systemic sclerosis. Hughes M, Pauling JD, Jones J, Denton CP, Domsic RT, Frech TM, Herrick AL, Khanna D, Matucci-Cerinic M, McKenzie L, Saketkoo LA, Gooberman-Hill R, Moore A. Hughes M. Rheumatology (Oxford). 2020 Aug 1;59(8):2156-2158. doi: 10.1093/rheumatology/keaa037.
   
   
4. Multicenter Qualitative Study Exploring the Patient Experience of Digital Ulcers in Systemic Sclerosis. Hughes M, Pauling JD, Jones J, Denton CP, Domsic RT, Frech TM, Herrick AL, Khanna D, Matucci-Cerinic M, McKenzie L, Saketkoo LA, Gooberman-Hill R, Moore A.Hughes M, et al. Among authors: Pauling jd. Arthritis Care Res (Hoboken). 2020 May;72(5):723-733. doi: 10.1002/acr.24127.
   
   
5. What narrative devices do people with systemic sclerosis use to describe the experience of pain from digital ulcers: a multicentre focus group study at UK scleroderma centres. Jones J, Hughes M, Pauling J, Gooberman-Hill R, Moore AJ.Jones J, et al. Among authors: pauling j. BMJ Open. 2020 Jun 11;10(6):e037568. doi: 10.1136/bmjopen-2020-037568.BMJ Open. 2020. PMID: 32532783
   
   
6. Exploring the patient experience of digital ulcers in systemic sclerosis.
   Hughes M, Pauling JD.Hughes M, et al. Among authors: Pauling jd. Semin Arthritis Rheum. 2019 Apr;48(5):888-894. doi: 10.1016/j.semarthrit.2018.08.001. Epub 2018 Aug 11.Semin Arthritis Rheum. 2019. PMID: 30205981
   
   
7. Pauling, J.D., et al., Patient-reported outcome instruments for assessing Raynaud’s phenomenon in systemic sclerosis: A SCTC Vascular Working Group Report. Journal of Scleroderma & Related Diorders, 2018. 3(3): p. 249-252.
   
   
8. Pauling, J.D., Saketkoo, L.A., Matucci Cerinic, M., Ingegnoli, F., Khanna, D., The patient experience of Raynaud’s phenomenon in systemic sclerosis. Rheumatology, 2018. ePub ahead of print.
   
   
9. Pauling, J.D., et al., Multi-national qualitative research study exploring the patient experience of Raynaud’s phenomenon in systemic sclerosis. Arthritis Care Res (Hoboken), 2018. 70(9): p. 1373-84.
   
   
10. Pauling, J.D., E.S. Reilly, T., and T. Frech, Evolving symptoms of Raynaud’s phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity. Arthritis Care & Research, 2018
    
    
11. Pauling, J.D., Saketkoo, L.A., Domsic, R.T., Patient perceptions of the Raynaud’s Condition Score diary provide insight into its performance in clinical trials of Raynaud’s phenomenon.” Arthritis & Rheumatology, 2018. 70(6): p. 973-4.
12. Hughes, M. and J.D. Pauling, Exploring the patient experience of digital ulcers in systemic sclerosis. Semin Arthritis Rheum, 2018.

Contact information for how members can get involved:

• Dr. John Pauling (John.Pauling@nbt.nhs.uk)