THU0349 THE RELATIONSHIP BETWEEN DISEASE ACTIVITY AND SEVERITY IN SYSTEMIC SCLEROSIS: A PROSPECTIVE ANALYSIS OF 278 PATIENTS

Abstract

Background:Evaluating disease activity and severity in systemic sclerosis (SSc) is crucial to define the patients who are candidate for treatment options.Objectives:We aimed to investigate the relationship between disease activity and severity in SSc in a large cohort.Methods:This is a cross-sectional prospective analysis of 278 (253 females) patients fulfilling ACR/EULAR (2013) classification criteria for SSc. Disease activity and severity were calculated seperately for cutaneous subsets (EscSG and Medsger). The patients were grouped as inactive if EscSG score=0, mildly active if EscSG score>0<3, active if EscSG score≥3.Results:The mean age, duration of Raynaud’s and non-Raynaud features were 48.5±13.1, 12.1±9.8 and 8.3±7.5 years respectively. Characteristics of the SSc patients were summarized in table-1.Ninety-three (34%) and 151(54%) patients were evaluated as having active and mildly active disease. Only 34(12%) patients had inactive disease. The patients with diffuse cutaneous involvement (dcSSc) who were active had higher modified Rodnan Skin score(mRSS) and severity scores of general, skin and joint-tendon involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin compared to those with inactive disease (table-2).The patients with limited cutaneous involvement (lcSSc) who were assessed as having active disease had higher mRSS and higher severity scores of general, skin, peripheral vascular, lung, joint-tendon and gastrointestinal involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin, lung, joint-tendon and gastrointestinal involvements compared to those with inactive disease (table-2).Conclusion:One third of our cohort was found to have active disease despite treatment and only 12% had inactive disease. Skin involvement and severity of different organs were shown to be higher in patients with active disease in both cutaneous subsets, together with severity of lung, peripheral vascular and gastrointestinal involvements in active lcSSc. LcSSc and dcSSc patients who had mildly active disease also had severe disease similar to those with active patients. Disease activity and severity should be assessed as separate measurements to highlight the course of the disease and may guide to the management of patients with SSc.Disclosure of Interests:None declared