PO:31:165 | Prevalence and incidence of skin telangectasia and their association with clinical features: analysis of the European Scleroderma Trials and Research cohort

Gemma Lepri¹, Lorenzo Tofani², Silvia Bellando Randone¹, Veronica Batani³, Paolo Airò⁴, Patricia Carreira⁵, Joerg Henes⁶, Alexandra Balbir Gurman⁷, Florenzo Iannone⁸, Antonella Marcoccia⁹, Luc Mouthon¹⁰, Branimir Anic¹¹, Carolina De Souza Müller¹², Armine Haroyan¹³, Ana Cristina Cordeiro¹⁴, Serena Guiducci And Eustar Coll¹. | ¹University of Florence, Dept of Experimental and Clinical Medicine, AOU Careggi, Division of Rheumatology Florence Italy; ²University of Florence, Dept of Statistics, Informatics, Applications, Florence, Italy; ³Unit of Immunology, Rheumatology, Allergy and Rare Diseases ^UnIRAR, IRCCS San Raffaele, Milano, Italy; ⁴Scleroderma Unit, UOC Rheumatology and Clinical Immunology, ASST Spedali Civili, Brescia, Italy; ⁵Dept of Rheumatology, 12 de Octubre University Hospital, Madrid, Spain; ⁶Centre for Interdisciplinary Rheumatology, Immunology and autoimmune diseases INDIRA, University Hospital Tuebingen, Tuebingen, Germany; ⁷Rheumatology Institute, Rambam Health Care Campus Haifa Israel; ⁸Dept of Precision and Regenerative Medicine and Ionian Area, University of Bari, Bari, Italy; ⁹Interdisciplinary Reference Center for Systemic Sclerosis ^CRIIS Sandro Pertini Hospital, Rome, Italy; ¹⁰Dept of Internal Medicine, Cochin Hospital, Paris Cité University, Paris, France; ¹¹Div Clinical Immunology and Rheumatology, Dept Internal Medicine, School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia; ¹²Rheumatology Division, Hospital de Clínicas da Universidade Federal do Parana, Curitiba, Brazil; ¹³Dept of Rheumatology, Yerevan State Medical University of Armenia, Yerevan, Armenia; ¹⁴Servico Reumatologia Hospital Garcia de Orta Almada, Almada, Portugal.

Background. Skin telangiectasias (TAs) are common in systemic sclerosis (SSc) and suggested to be associated with a more severe vascular phenotype. The aim of our work was to analyse prevalence and incidence of TAs in a SSc multicentric cohort and their clinical correlations.

Materials and Methods. SSc patients from the European Scleroderma Trials and Research (EUSTAR) database with baseline data on TAs were enrolled to analyse the prevalence and TAs and their clinical correlations (project CP156). During the follow-up we evaluated the incidence of TAs and compared disease features between patients with TAs from baseline, those developing TAs during the follow-up and those without.

Results. A total of 8.088 patients with baseline data on TAs, of whom 4.050 presented TAs with a prevalence of 50.1% (IC 0.49-0.51). At baseline patients with TAs showed significant differences in disease phenotype compared to those without (Fig 1): a greater skin involvement (8.3±8.6 vs 7.0±7.8, p:<0.0001) and higher mean NT-proBNP values (456.4±1833.7 vs 304.4±2114.0, p:<0.0001) were observed in patients with TAs that also presented higher frequency of anticentromere antibodies (ACA) and lower prevalence of anti-topoisomerase I (Topo I) (p<0.0001 and 0.0278 respectively). Puffy fingers were more frequent in patients with TAs (1747 vs 1573, p:0.0147) and a significantly higher prevalence of digital ulcers (DUs) and digital pitting scars (DPS) (current or previous) was observed in TAs patients. The mean values of DLCO and DLCO/VA were lower in SSc patients with TAs (67.8±20.3 vs 73.8±20.5 and 76.3±18.3 vs 82.2±17.8 respectively, p<0.001) that also presented a higher frequency of ILD at HRCT (1293 vs 1017, p:<0.001). Echocardiographic abnormalities were more prevalent in TAs patients. The incidence of TAs was of 32.6% (IC 0.31-0.34) in a median observation time of 1.61 years (1.50-1.74) (Figure 2). The median follow-up time was of 1.02 years (0.701-1.56) for patients with TAs from baseline (group1: 4050 patients), 1.61 (1.01-3.01) for those developing TAs (group2: 1313 patients) and 2.78 (1.29-5.15) for those without TAs (group3: 2716 patients). Comparing disease features between the three populations at the end of follow-up we confirmed some of the data reported at baseline (Fig 3): patients with TAs from baseline or developing TAs during the follow-up had a more sever skin involvement, a greater prevalence of DUs, DPS, subcutaneous calcinosis, tendon friction rubs, joint involvement, ILD and also at the end of follow-up they presented lower LFTs parameters.

Conclusions. Our data clearly show that TAs are associated with a more aggressive SSc phenotype from both a cardiovascular and pulmonary point of view. Our data have to be confirmed by future studies also evaluating whether the presence of TAs represents a risk factor for the development of organ complications.