Damage & Activity Index Working Group

The SCTC supports a working group to develop a disease Activity Index in systemic sclerosis. This instrument has been developed to systematically identify and quantify potentially reversible disease activity. The applications of this Activity Index include use as an outcome measure in observational and interventional studies. The development of the Activity Index is a continuation of work undertaken by the same group of investigators who worked to develop a disease Damage Index in scleroderma.

SCTC Working Group Summary

Projects

SCTC Damage Index

Work on development of the SCTC DI has now been completed, with a manuscript soon to be submitted for peer review publication.

SCTC Activity Index

The goals of this working group are to:

Develop a consensus conceptual definition of the construct of disease activity in systemic sclerosis
Using a combination of consensus and data-driven methodology, develop a multi-system, weighted disease activity index.

Progress to date:

Following a two-round survey and face-to-face meeting at last year’s SCTC AGM, the following conceptual definition of disease activity in systemic sclerosis has been proposed:
Disease activity in systemic sclerosis refers to aspects of disease, attributable to systemic sclerosis, that are potentially reversible, or can be arrested, with time and/or effective therapy. Disease activity may be associated with morbidity and uncontrolled activity may lead to organ dysfunction and mortality.
In order to generate items for potential inclusion in the activity index, working group members have initially been surveyed as to their opinion of the various domains of disease that should be included in the activity index. Using the results of this initial survey, the working group has then been asked their opinion of various individual items to measure activity within each domain and also to generate additional potential items for inclusion in the activity index.
The results of this second survey was discussed at the AGM in Chicago (2018) and the next steps required to finalize activity items will be discussed.
A provisional, weighted index is expected to be presented to the working group at the AGM in 2019.

Publications:

Baron, M., Kahaleh, B., Bernstein, E. J., Chung, L., Clements, P. J., Denton, C… Vacca, A. (2018). An interim report of the Scleroderma Clinical Trials Consortium working groups. Journal of Scleroderma and Related Disorders. https://doi.org/10.1177/2397198318783926