Systemic Sclerosis - Disease Severity and Activity Assessment

Overview

The authors present an overview of scleroderma classification as a whole, classification criteriaof systemic sclerosis (SSc) and its subclassification into two subsets: limited and diffuse. Variabilityof SSc clinical course, early organ dysfunction of diffuse subset, difficulty of the disease activityassessment need standardized tools for monitoring the disease severity (the disease status), whichare necessary for predicting prognosis of an individual patient and for evaluation of clinical trials.The disease severity at a given time represents combination of tissue damage, i.e. irreversible organdysfunction, and disease activity, i.e. potentially reversible organ dysfunction. The preliminarydisease severity criteria evaluating nine organ systems in 4-grade-scale recommended for use inclinical trials by European Scleroderma Study Group, ESSG, are reviewed. Also the preliminaryclinical SSc activity criteria identified in multicenter clinical study designed by the same group,which are validated in another ongoing study organized by recently founded European groupEUSTAR, i.e. EUlar (European League Against Rheumatism) Scleroderma Trials And Research, arepresented. The list of “core set“ and special potential serological markers of the disease activityderived from the pathogenesis of SSc, which were selected for clinical trials by ESSG, are included.

Key words:
scleroderma - systemic sclerosis - classification - severity - activity