{
  "abstract": "Introduction The aim of our work was to analyse prevalence and incidence of TAs in a SSc multicentric cohort and their clinical correlations.Material and Methods SSc patients from the 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 8.088 patients had baseline data on TAs and 4.050 presented TAs with a prevalence of 50.1% (IC0.49-0.51). At baseline patients with TAs showed differences in disease phenotype compared to those without ( figure 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 also presenting higher frequency of anticentromere antibodies and lower prevalence of anti-topoisomerase 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) 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% (IC0.31-0.34) 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 (figure 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 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.Abstract P.376 Figure 1Disease features at haseline in the two populations (patierts with TAs and patients without)Abstract P.376 Figure 2Time to TAs appearanceAbstract P.376 Figure 3Disease features at follow-up in the three populctions",
  "authors": [
    {
      "affiliations": [
        "University of Florence, Dept of Experimental and Clinical Medicine, AOU Careggi, Division of Rheumatology, Florence, Italy"
      ],
      "name": "Gemma Lepri"
    },
    {
      "affiliations": [
        "University of Florence, Dept of Statistics, Informatics, Applications, Florence, Italy"
      ],
      "name": "Lorenzo Tofani"
    },
    {
      "affiliations": [
        "University of Florence, Dept of Experimental and Clinical Medicine, AOU Careggi, Division of Rheumatology, Florence, Italy"
      ],
      "name": "Silvia Bellando-Randone"
    },
    {
      "affiliations": [
        "Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele, Milan, Italy"
      ],
      "name": "Veronica Batani"
    },
    {
      "affiliations": [
        "Scleroderma Unit, UOC Rheumatology and Clinical Immunology, ASST Spedali Civili, Brescia, Italy"
      ],
      "name": "Paolo Airò"
    },
    {
      "affiliations": [
        "Dept of Rheumatology, de Octubre University Hospital, Madrid, Spain"
      ],
      "name": "Patricia Carreira"
    },
    {
      "affiliations": [
        "Centre for Interdisciplinary Rheumatology, Immunology and autoimmune diseases (INDIRA), University Hospital Tuebingen, Tuebingen, Germany"
      ],
      "name": "Joerg Henes"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Haifa, Israel"
      ],
      "name": "Alexandra Balbir Gurman"
    },
    {
      "affiliations": [
        "Dept of Precision and Regenerative Medicine and Ionian Area, University of Bari, Bari, Italy"
      ],
      "name": "Florenzo Iannone"
    },
    {
      "affiliations": [
        "Interdisciplinary Reference Center for Systemic Sclerosis (CRIIS) Sandro Pertini Hospital, Rome, Italy"
      ],
      "name": "Antonella Marcoccia"
    },
    {
      "affiliations": [
        "Dept of Internal Medicine, Cochin Hospital, Paris Cité University, Paris, France"
      ],
      "name": "Luc Mouthon"
    },
    {
      "affiliations": [
        "Div Clinical Immunology and Rheumatology, Dept Internal Medicine, School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia"
      ],
      "name": "Branimir Anic"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital de Clínicas da Universidade Federal do Parana, Curitiba, Brazil"
      ],
      "name": "Carolina De Souza Müller"
    },
    {
      "affiliations": [
        "Dept of Rheumatology, Yerevan State Medical University of Armenia, Yerevan, Armenia"
      ],
      "name": "Armine Haroyan"
    },
    {
      "affiliations": [
        "Servico Reumatologia Hospital Garcia de Orta Almada, Almada, Portugal"
      ],
      "name": "Ana Cristina Cordeiro"
    },
    {
      "affiliations": [
        "University of Florence, Dept of Experimental and Clinical Medicine, AOU Careggi, Division of Rheumatology, Florence, Italy"
      ],
      "name": "Serena Guiducci Eust"
    }
  ],
  "title": "P.376 Prevalence and incidence of skin telangectasia and their association with clinical features: analysis of the European scleroderma trials and research (EUSTAR) cohort",
  "uid": "61f0519d-d1c8-59fc-8f80-6b05fc21be10"
}