
Objectives: The scleroderma interest group of ``The Society for Research and Education in Rheumatology (RAED-Turkey)'' formed a database (Sys-Watch) for patients with systemic sclerosis (SSc) to collect clinical and laboratory information from rheumatology centers as a basis for prospective studies. The aims of Sys-Watch are education of the centers according to EUSTAR principles, establishing a platform for research and using a uniform protocol for the follow-up of the patients with scleroderma with a prospect of outcome analysis.
Methods: Sys-Watch protocol includes demographic, clinical and laboratory features of consecutive SSc patients attending to the rheumatology centers across the country. Additionally, values for modified Rodnan skin score, disease activity index(1), visual analog scale for disease activity for both doctors and patients, severity scale (2) and functional score (3) were collected prospectively. A total of 900 patients from 25 centers were recruited for the study.
Results: Among 900 patients, 91.2% was female and the mean age of the patients was 49.9 years. Mean disease duration was 11.8 years. All patients fulfilled the ACR preliminary criteria for SSc. Forty two percent of the patients had diffuse cutaneous involvement. More than 12% had autoimmune disease and 2.7% had SSc in the family. The prevalence of Raynaud's was 93%, digital ulceration 39%, gangrene 9%, arthritis 22%, renal crisis 3%, Dysphagia 49%, dyspnea 48%, heart conduction defects 2%, arrythmia 4%, reduced FVC 31%, reduced DLCO 40%, lung fibrosis 39%. ANA positivity was found in 78% anti-scl70 48% and, anti-centromere 43%. Echocardiographic assessment was done in 831 patients and 10% had elevated pulmonary arterial pressures (>40mmHg). In 37% of the patients disease activity score was 3 or more. Selected organ systems of severity scale were given in the Table.According to the functional scale 81% had some degree of functional impairment with a mean score of 7.5/33. Correlation analysis revealed that disease activity index had moderate correlation with the functional scores (r=0.45), VAS (patient) (r=0.49), and VAS(dr) (r=0.61)(all p<0.001).
SSc severity scale of the SysWatch cohort in percentages
Organ System Normal Mild Moderate Severe Endstage Peripheral Vascular 7 45 29 15 4 Joint/Tendon 57 12 22 5 4 Lung 33 34 21 11 1 Skin 6 50 35 7 2 Gastrointestinal Tract 46 48 4 1 1
Conclusion: Gastrointestinal system, cardiopulmonary system an peripheral vascular system were the more frequent organ involvements in this cohort and a very low frequency of renal crisis was noted. Peripheral vascular and lung involvement were relatively severe. More than 30% of the patients had active disease by using disease acitivity index and most of the patients had functional impairment.
References:
1. Valentini G, et al. Ann Rheum Dis 2001;60:592
2. Medsger T, et al. J Rheumatol 1999;26:2159
3. Silman A, et al. J Rheumatol 1998;25:79
Disclosure of Interest: None declared