{
  "abstract": "Introduction The prevalence of SSc ranges from 31 per million in Japan to 658 per million in Choctaws native American tribe in Oklahoma. The aim of the study was to determine the prevalence of SSc in Israel, to identify geographic hotspots and to understand whether these are related to environmental factors.Material and Methods We used the Clalit Health Services (CHS) database that includes 4.8-million-members. Adult SSc patients were identified between 2005-2023 using ICD-9 diagnosis codes of SSc at least two separate records 6 months apart in the presence of serological confirmation. We created a prevalence heat map and further assessed spatial autocorrelation using Global Moran’s I and subsequently Local Moran’s I (LISA) was applied to identify high-risk ‘hotspots’. We further applied spatial Poisson regression with population at risk as the offset, modeling pollutant exposure in quartiles.Results A total of 3,385 patients met the criteria for SSc ( table 1), of which 817 (24%) were positive for Scl-70, 821 (24%) for ACA, 416 (12%) for RNA POLII, and 1,331 (40%) were only ANA positive. 87% were females, mean age at diagnosis was 54+/-16 (table 1). The incidence of SSc was in average 3.85 per 100,000 person-years. The mean prevalence of SSc was 633 per 1 million but reached over 1000 per 1 million. Five-year survival was 86.4% to 89.7%, and 10-year survival were 76.6% to 80.6 % with no differences between groups. The standardized mortality rate (SMR) was =3.41 [95%CI: 3.15-3.67].Autoantibodies did predict survival in a COX regression analysis (log rank p=0.93) (figure 1a).Conclusions We have found a Strikingly high prevalence of SSc, 633 per 1 million in Israel, comparable to the highest prevalence reported in the world in Choctaws native American in Oklahoma, largely attributed to a unique HLA haplotype. The high prevalence clusters were associated with NOx pollution. Heterogenous populations in Israel including Sephardic and Ashkenazi and Ethiopian Jews, Arab, Druze and Bedouin may help explain the high prevalence of SSc. Specifically, high prevalence in Druze villages should be further explored regarding a genetic factor.Conclusions Initial therapy was associated with improved survival and reduced PAH progression in SSc-PAH, with consistent effects across haemodynamic thresholds and risk strata. These findings support guideline-recommended early intervention, highlight the importance of high-quality observational data in rare diseases, and underscore the need for RCTs to clarify treatment effects in patients with milder haemodynamic impairment.Abstract OC.15 Table 1Abstract OC.15 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit, Bnai Zion Medical Center, Faculty of Medicine, Technion Israel institute of technology, Haifa, Israel"
      ],
      "name": "Doron Rimar"
    },
    {
      "affiliations": [
        "Faculty of medicine, Ben-Gurion University of the Negev, Beer-Sheva, Israel"
      ],
      "name": "Ilan Libes"
    },
    {
      "affiliations": [
        "Research Unit, Emek Medical Center, Afula, Israel"
      ],
      "name": "Snait Ayalon"
    },
    {
      "affiliations": [
        "Rheumatic Diseases Unit, Emek Medical center, Technion Israel Institute of Technology, Rappaport Faculty of Medicine, Afula, Israel"
      ],
      "name": "Shay Brikman"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Bnai Zion Medical Center, Faculty of Medicine, Technion Israel institute of technology, Haifa, Israel"
      ],
      "name": "Shiri Keret"
    },
    {
      "affiliations": [
        "Research Unit, Emek Medical Center, Afula, Israel"
      ],
      "name": "Naama Schwartz"
    },
    {
      "affiliations": [
        "Rheumatic Diseases Unit, Emek Medical center, Technion Israel Institute of Technology, Rappaport Faculty of Medicine, Afula, Israel"
      ],
      "name": "Amir Bieber"
    }
  ],
  "title": "OC.15 Systemic sclerosis in Israel: a 20-year nationwide study of prevalence, hot spots and survival analysis",
  "uid": "fa709078-5936-525e-b941-392f41bf2fce"
}