THU0369 EVALUATION OF DIFFERENT CLASSIFICATION CRITERIA IN SYSTEMIC SCLEROSIS IN A TURKISH COHORT: THE IMPORTANCE OF NON-SKIN MANIFESTATIONS, SEROLOGY AND CAPILLAROSCOPY
Authors: Y. Yalçinkaya, S. Amikishiyev, N. Aliyeva, B. Artim-Esen, A. Gul, L. Ocal, M. Inanc
Journal: Annals of the Rheumatic Diseases (2020)

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 SSc ACR/EULAR (2013) + ACR/EULAR (2013 )- total ACR (1980)+ 108 0 108 ACR (1980 )- 19 12 31 total 127 12 139 Table 2. SSc patients fulfilling ACR (1980) and/or ACR/EULAR (2013) criteria ACR/EULAR (2013) ACR (1980 ) Classified n=127 Not classified n=12 +Prox scleroderma + 87 +Puffy hands 29 4 +Sclerodactyly + 112 1 +Digital ulcers + +Pitting scars 62 72 +Telangiectasia 86 2 +Nailfold capillaroscopy 97 4 Normal 9 Early 17 3 Active 40 Late 50 1 +PAH 12 +Interstitial lung Disease + 60 +Raynaud’s 120 12 +SSc- specific antibodies 79 5 Conclusion: 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 &gt;%96 for both criteria, in limited cutaneous subgroup, the sensitivity was preserved for ACR/EULAR(2013) while apparently decreased for ACR(1980) criteria (&lt;%70). The sensitivity for both of the two sets were lowest in the asclerodermic group. In SSc patients with limited or no skin involvement, non-skin manifestations, NVC findings and spcific serology should be carefully sought. Some of these patients could not be classified by the current criteria. Disclosure of Interests: None declared