Measures of disease status in systemic sclerosis: A systematic review

To identify and appraise measures of disease status in systemic sclerosis (SSc).

A systematic review of Medline (1966-2015), EMBASE (1974-2015), and Cochrane Library (inception-2015) was undertaken to identify indices of disease status in SSc. We focused on objective measures and excluded non-English articles. Measures were reviewed for content, whether they measured activity, damage and/or severity and whether they were validated according to the OMERACT filter.

Of the 4558 articles retrieved through the search, we identified 58 articles for review. We found a further 44 articles through a search of the bibliography of relevant articles. We identified the following 10 "composite" (multi-organ) indices: two disease activity indices, six disease severity scales, and two combined response indices. There was no disease damage index for SSc.

We identified a number of composite and organ-specific indices in SSc, incorporating mostly objective measures, developed to quantify disease activity, severity, and response in clinical trials. However, none of the indices was developed to exclusively quantify organ damage. Most of the existing indices require further validation according to the OMERACT filter. There is a need to develop and validate a disease damage index in SSc.