A disease severity scale for systemic sclerosis: development and testing.

摘要: Objective To develop and test a severity scale for individual organ involvements in systemic sclerosis (SSc, scleroderma). Methods An international study group completed the following tasks: (1) developed glossary of terms including all pertinent variables 9 potentially affected systems; (2) collected prospective data to determine feasibility practicality each proposed variable; (3) revised initial list variables; (4) determined association variable with mortality (a proxy morbidity) using 579 patients an existing comprehensive longitudinal scleroderma databank; (5) grading system by discussion consensus; (6) externally validated independent 680 from same databank. Results Nine organ-specific scales were 0 (no documented involvement) 4 (endstage disease). The required completion are relatively easy practical physicians obtain. Conclusion This preliminary will be useful assessing disease status both at one point time longitudinally. assist design conduct clinical trials comparison populations another. serve as framework developing activity index.