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Background: Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder with high morbidity and mortality rates. SSc-associated autoantibodies are important diagnostic and predictive biomarkers of distinct clinical subtypes and internal organ manifestations. Despite variable antibody prevalence between different geographic regions, their clinical associations remain consistent. Objectives: The aim of this study was to assess the clinical and immunological features of a South-Italy multicenter SSc cohort. Methods: A retrospective cohort study of 895 SSc patients was conducted. Clinical and laboratoty data was collected from unique database of Campania and analyzed by means of descriptive and inferential statistics (Chi-square test for Indipendence, Relative Risk/RR). In this study la prevalence of antinuclear (ANA), anti-topoisomerase (anti-TOPO I/anti-Scl70) and anti-centromere (ACA) antibodies was evaluated, as well as their association with specific clinical phenotypes and organ system involvement. Results: 2013 ACR/EULAR classification criteria were fulfilled in all case. The mean of follow-up was 96.5 months. Female to male ratio was 7:1; with 68% with limited-SSc (lcSSc) vs 32% diffuse (dcSSc). ANA were negative in 1 patient. The most frequently autoantibodies found was ACA (57%), followed by anti-Scl-70 (35%). A significant relationship between anti-TOPO I and dcSSC was identified (pConclusion: this study acknowledges the strong association between anti-TOPO-I and dcSSc, as well as relationship between ACA and lcSSc. Anti-TOPO-I is a known risk factor for the development of progressive lung fibrosis and cardiac involvement and therefore these patients should be actively screened for interstitial pulmonary involvement and cardiac dysfunction REFERENCES: NIL. Acknowledgements: Lega Italiana Sclerosi Sistemica. Disclosure of Interests: None declared.
Cuomo et al. (Sat,) studied this question.