LAUSR

OBJECTIVES to comprehensively identify instruments within relevant domains employed to assess limited cutaneous systemic sclerosis (lcSSc) since the endorsement of its consensus definition in 1988. The overall objective is to inform the creation of a combined response index for lcSSc (Combined response index for scleroderma trial assessing limited scleroderma (CRISTAL)). METHODS MEDLINE and EMBASE were searched with terms selected to comprehensively retrieve titles/abstracts mentioning both lcSSc and dcSSc along with those only mentioning lcSSc, SSc sine scleroderma, limited SSc and/or CREST/CRST. Because our initial assessment of the literature revealed that very few studies included only lcSSc subjects, we assessed literature that included either only lcSSc or both cutaneous subsets. 3964 Titles and Abstracts were screened by two reviewers. 270 articles were selected for data extraction. RESULTS We identified 27 domains encompassing 459 instruments. Instruments from "Skin involvement", "Pulmonary involvement" and "Health-related quality of life and general functioning" were the most frequently retrieved. Among the 15 most represented instruments announced as primary endpoints in efficacy or effectiveness studies, 7 were Clinician Reported Outcomes (RO), 7 were patient ROs and one was a Performance outcome (6 min-walk test). The mean proportion of lcSSc patients in studies including both lc and dcSSc, was 56.4% demonstrating that this subset is underrepresented in the literature. CONCLUSION This scoping literature review provides a comprehensive identification of domains and outcomes used to assess lcSSc. Our results also highlight that lcSSc is under-represented in the literature.