The SCTC SRC Working Group, consisting of key international experts from rheumatology, nephrology and pathology was created in November 2015 to develop and validate classification criteria for scleroderma renal crisis (SRC). A scoping review to generate evidence-based items to define SRC was performed during 2016, presented to the SCTC SRC working group in Washington, DC, in November 2016, and published in February 2017 (1). In May-October 2017, we conducted a 3-round online Delphi exercise to develop initial consensus on a core set of items to define SRC that had face and content validity, and were scientifically valid and feasible. In November 2017, a nominal group discussion (face-to-face structured group meeting of experts) was held in San Diego, CA, to achieve final consensus on the core set. Considerable input was also sought from content experts in nephrology, hematology, neurology, ophthalmology, cardiology and pathology. Final consensus was achieved on items for blood pressure, acute kidney injury, microangiopathic hemolytic anemia and thrombocytopenia, target organ dysfunction (cardiac dysfunction, retinopathy and encephalopathy) and histopathology. A manuscript reporting the results of the Delphi exercise and NGT meeting is currently under review (Arthritis Rheumatol).The SCTC SRC Working Group, consisting of key international experts from rheumatology, nephrology and pathology was created in November 2015 to develop and validate classification criteria for scleroderma renal crisis (SRC). A scoping review to generate evidence-based items to define SRC was performed during 2016, presented to the SCTC SRC working group in Washington, DC, in November 2016, and published in February 2017 (1). In May-October 2017, we conducted a 3-round online Delphi exercise to develop initial consensus on a core set of items to define SRC that had face and content validity, and were scientifically valid and feasible. In November 2017, a nominal group discussion (face-to-face structured group meeting of experts) was held in San Diego, CA, to achieve final consensus on the core set. Considerable input was also sought from content experts in nephrology, hematology, neurology, ophthalmology, cardiology and pathology. Final consensus was achieved on items for blood pressure, acute kidney injury, microangiopathic hemolytic anemia and thrombocytopenia, target organ dysfunction (cardiac dysfunction, retinopathy and encephalopathy) and histopathology. A manuscript reporting the results of the Delphi exercise and NGT meeting is currently under review (Arthritis Rheumatol).

Two additional components of this research project are now in the planning phases. First, modeled on the first International Scleroderma Renal Crisis Survey (ISRCS) (2), ISRCS II will be developed and launched to collect a new inception SRC cohort to validate the consensus criteria using data-driven methods. Second, a forced choice study using multi-criteria decision analysis methods(3) will be performed with the experts who participated in the nominal group discussion to assign weights to the items in the criteria. ISRCS II is expected to take approximately 4 years (2 years of baseline data collection, 1 year of follow up data collection and 1 year of data analysis). The forced choice experiment will be conducted during this time.

There is an increasing number of clinical trials in SSc, many in early diffuse disease. These represent unique opportunities to identify and study SRC. In addition, novel therapies for SRC will require testing in clinical trials. This project will generate the first validated classification criteria for SRC. These criteria are expected to become the international standard and will be used in future randomized trials and epidemiologic research of SSc.

Contact information:

Dr Marie Hudson, MD MPH

Jewish General Hospital, Lady Davis Institute for medical research, 3755 Côte Ste Catherine, Montreal, QC, H3T 1E2, Canada

Email: [email protected]

Dr Christopher Denton, MD PhD

Centre for Rheumatology, Royal Free Campus, Rowland Hill Street, London, NW3 2PF, UK

Email: [email protected]