Renal Crisis Working Group

Summary of Current Projects:

• The SCTC Scleroderma Renal Crisis Working Group was created to develop and validate classification criteria for scleroderma renal crisis (SRC).

Progress to Date:

• In Phase 1, a scoping review to generate evidence-based items to define SRC was performed [1].
• In Phase 2, we undertook item reduction using consensus techniques (3-round online Delphi exercise and Nominal group discussion) to generate a core set of items to define SRC [2].
• An inception cohort of SRC cases was recruited internationally to test the extent to which the consensus-derived core set captured the spectrum of SRC using real-world data, to determine one-year outcomes and to investigate treatment patterns for SRC [3].

• In Phase 3, we conducted a multi-criteria decision analysis exercise using the 1000Minds software to weight core items and produce a provisional threshold score to classify a case as SRC.

• In Phase 4, we are collecting a large international cohort of SRC cases and controls to 1) refine the weights and threshold score in a new derivation cohort, and 2) test the sensitivity and specificity of the final criteria in an independent validation cohort.  

Next Steps:

• Complete the collection of SRC cases and controls in Phase 4
• Conduct independent adjudication of the SRC cases and controls collected in Phase 4
• Test the performance of the draft criteria set produced in Phase 3 and determine a threshold for classification in a derivation cohort, which will consist of approximately half of the cases and controls collected in Phase 4.
• Test the sensitivity and specificity of the final criteria in a validation cohort, which will consist of the other half of the cases and controls collected in Phase 4.

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

• Produce the first expert- and data-driven classification criteria for SRC – end of 2026

• Draft and submit a manuscript for peer review – end of 2026/beginning of 2027

Publications:

[1] Hoa S, Stern EP, Denton CP, Hudson M, Scleroderma Clinical Trials Consortium Scleroderma Renal Crisis Working Group Investigators of the Scleroderma Clinical Trials Consortium Scleroderma Renal Crisis Working G. Towards developing criteria for scleroderma renal crisis: A scoping review. Autoimmun Rev. 2017;16(4):407-15. [PMID: 28212921]

[2] Butler EA, Baron M, Fogo AB, Frech T, Ghossein C, Hachulla E, Hoa S, Johnson SR, Khanna D, Mouthon L, Nikpour M, Proudman S, Steen V, Stern E, Varga J, Denton C, Hudson M, Scleroderma Clinical Trials Consortium Scleroderma Renal Crisis Working Group. Generation of a core set of items to develop classification criteria for Scleroderma renal crisis using consensus methodology. Arthritis Rheumatol. 2019;71(6):964-71. [PMID: 30614663]

[3] Chan R, Baniña M, Berger C, Ghossein C, Hoa S, Johnson SR, Shen HC, Stern EP, Denton CP, Hudson M, Investigators of the International Scleroderma Renal Crisis Survey II, on behalf of the Scleroderma Clinical Trials Consortium Scleroderma Renal Crisis Working Group. Treatment Patterns and Outcomes of Scleroderma Renal Crisis in the International Scleroderma Renal Crisis Survey II – An Inception Cohort Study. (submitted)

Contact information for how members can get involved:

• Dr. Marie Hudson (marie.hudson@mcgill.ca)
• Dr. Christopher Denton (c.denton@ucl.ac.uk)