AB0752 Clinical features of systemic sclerosis associated to anti rna polymerase iii antibodies in valld’hebron hospital
Authors: E.L. Callejas-Moraga, A. Guillén-Del-Castillo, A. Marín-Sánchez, V. Fonollosa-Plá, E. Balada, J. Martín, C.P. Simeón-Aznar
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Objectives</h3> Systemic sclerosis (SSc) is an autoimmune disease whose clinical features are related closely with the specific autoantibody that is expressed. The main objective was to analyse the clinical characteristics of patients with positive anti-RNA polymerase III antibody (anti-RNAP III) and compare them with patients who were negative. <h3>Methods</h3> We analysed 221 patients with SSc who visited Valld'Hebron Hospital from 1980 to 2016. 26 patients who were positive were compared with 195 who were negative. Different epidemiological, clinical, capillaroscopic and immunological variables were analysed. Data from the Vall d'Hebron Hospital cohort were collected from patients diagnosed with ES since 1980. 24 patients with positive anti-RNAP III antibodies were selected using the technique of immunoblot on paper or enzyme immunoassay and were compared with 151 who were negatives. Different epidemiological, clinical, capillaroscopic and immunological variables were analysed. Statistical analysis was performed using SPSS 20.0. Statistical significance was considered p&lt;0.05. <h3>Results</h3> 24 patients (13.7%) showed anti-RNAP III reactivity. Female sex (151, 86.3%) was the most frequent. There were no differences in the age at onset of the disease (40.3±15.9) or in the age at diagnosis (46.6±15.7). Diffuse cutaneous (Dc) subtype was more frequent in the anti-RNAP III group (70% vs 16.6% p&lt;0.001). Patients with positive anti-RNAP III met the ACR/<sup>EULAR 2013</sup> diagnostic criteria more frequently (24, 100% vs. 118, 78.1% p&lt;0.01). Regarding the first manifestation, Raynaud's phenomenon was less frequent in the anti-RNAP III group (13, 54.2% vs. 121, 80.1% p&lt;0.01). Regarding to vascular features, there were no differences in digital ulcers (15, 62.5% vs. 66, 43.7%, p=0.08), in the presence of Raynaud's phenomenon (24, 100% vs. 145, 96.7%) or telangiectasias (18, 75% vs. 102, 67.5%). There were no differences with respect to the overall digestive involvement (21, 87.5 vs. 124, 82.1%) No differences were found in cardiac involvement in both groups (9, 37.5% vs. 51, 47%). Scleroderma renal crisis (SRC) was significantly more frequent in patients with anti-RNAP III (3, 12.5% vs. 3, 1.9% p=0.03). There were no differences in prevalence of cancer (4, 16.7% vs. 12, 7.9% p=0.24). There were no differences in mortality (2, 8.3% vs. 16, 10.6%). The slow-type capillary pattern of Maricq was the most frequent in both groups (20, 83.3% vs. 132, 87.4%). Regarding the expression of other autoantibodies, anti-Ro52 positivity was less frequent in patients with anti-RNAP III (2, 8.2% vs. 14, 9.3% p=0.03). <h3>Conclusions</h3> Patients with ES who presented positivity for anti-RNA polymerase III antibodies had more frequent onset manifestations different from the Raynaud's phenomenon, more DcSSc subtype and higher frequency of SRC. Regarding positivity against other antibodies, they expressed less reactivity to anti-Ro 52 than patients without anti-RNA pol III antibodies. <h3>Disclosure of Interest</h3> None declared