THU0369 EVALUATION OF DIFFERENT CLASSIFICATION CRITERIA IN SYSTEMIC SCLEROSIS IN A TURKISH COHORT: THE IMPORTANCE OF NON-SKIN MANIFESTATIONS, SEROLOGY AND CAPILLAROSCOPY

Background:Proximal scleroderma is the major criterion in both 1980 and 2013 classification criteria for sytemic scleroris (SSc). ACR(1980) criteria included digital lesions and bibasiler fibrozis, nonetheless ACR/EULAR(2013) criteria based on a scoring system including digital lesions, telangiectasia, abnormal nailfold video-capillaroscopy(NVC), PAH, Raynaud’s and specific autoantibodies.Objectives:We aimed to implement both criteria in a Turkish SSc kohort to evaluate the contribution of non-skin manifestations, NVC and autoantibodies.Methods:A consecutive hundred and thirty-nine (125 females) SSc patients diagnosed and evaluated by the same experts (YY, MI) with relevant NVC records and at least 6 months follow-up were included into the study. Classificaiton criteria were used retrospectively using a preformed database.Results:Characteristics of the SSc patients were summarized in table-1. The mean age, duration of Raynaud’s and non-Raynaud symptoms were 47.1±11.9, 8.9±7.9 and 5.7±5.8 years, respectively. Diffuse and limited cutaneous disease were diagnosed in 62(44.6%) and 60(43.2%) patients respectively. Asclerodermic disease was present in 17(12.2%) patients. ANA, anti-centromere and anti-Scl70(+) positivity was 80.5%, 18.0% and 37.4%, respectively.Twelve patients (8.6%) could not be classified as SSc by both criteria; 5 with Raynaud’s+specific antibodies (2 anti-centromere+, 2 anti-Scl70+), 4 with Raynaud’s+puffy hands+NC abnormalities, 2 with Raynaud’s+telangiectasia and a patient with Raynaud’s+sclerodactyly. Nineteen (13.7%) patients could not be classified as SSc according to ACR (1980) can be classsified according to ACR/EULAR (2013) (table-1 and -2).The sensitivity for ACR/EULAR (2013) and ACR (1980) criteria were found to be 91,4% vs 75,5%; 98.4% vs 96.8% in diffuse cutaneous SSc, 98.3% vs 68.3% in limited cutaneous SSc and 47.1 vs 23.5% in asclerodermic SSc, respectively.Table 1.The sensitivity for ACR (1980) and ACR/EULAR (2013) classification criteriain SScACR/EULAR (2013) +ACR/EULAR (2013)-totalACR (1980)+1080108ACR (1980)-191231total12712139Table 2.SSc patients fulfilling ACR (1980) and/or ACR/EULAR (2013) criteriaACR/EULAR (2013)ACR (1980)Classified n=127Not classified n=12+Prox scleroderma+87+Puffy hands294+Sclerodactyly+1121+Digital ulcers + +Pitting scars62 72+Telangiectasia862+Nailfold capillaroscopy974 Normal9 Early173 Active40 Late501+PAH12+Interstitial lung Disease +60+Raynaud’s12012+SSc- specific antibodies795Conclusion:The sensitivity of ACR/EULAR (2013) criteria was shown to be higher than ACR (1980) criteria in our Turkish SSc cohort with established cases. Although in diffuse cutaneous subgroup, the sensitivity was >%96 for both criteria, in limited cutaneous subgroup, the sensitivity was preserved for ACR/EULAR(2013) while apparently decreased for ACR(1980) criteria (Disclosure of Interests:None declared