{
  "abstract": "Introduction Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of 3 in 1 000 000 children. In adult patients antic-scl 70 antibodies correlate with the diffuse subtype and a specific organ involvement pattern. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects multinational data including clinical presentation, antibody profile and patient and physician reported outcomes.Material and Methods The jSScC enrolls jSSc patients who developed the first non-Raynauds symptom before the age of 16 years and are under the age of 18 years at the time of inclusion. We extracted the data at the time of inclusion in the registry from patients in the jSScC, including clinical manifestations and patient and physician reported outcomes until 15th of December 2024. Unknown antibody status for anti-Scl70 antibodies was an exclusion criteria.Results 260 patients from the cohort. 31% (n=80) were anti Scl-70 positive (antiScl70+). the anti-Scl70+ and anti-Scl70-group were similar regarding disease duration at time of inclusion (approximately 2.5 years), mean age at first non-Raynauds involvement (11 years of age), ethnicity, subtype of SSc and positive for anti-centromere antibodies. C-reactive protein (CRP) was elevated in 23% in the anti-Scl70+ group compared to 5% in the anti-Scl70- group (p <0.001). Anti-Scl70+ patients had less overlap features (18% vs 30%, p<0.001). Patients with anti-Scl70+ had higher rate of nailfold capillary changes (84% vs 74%, p=0.015), history of ulcerations (61% vs 47%, p=0.345) and telangiectasia (29% vs 40%, p=0.049). There were no differences regarding number of patients with decrease FVC<80%, DLCO<80% or positive HRCT findings for interstitial lung disease. Rate of pulmonary hypertension was 8% in anti-Scl70+ compared to 5% in anti-Scl70-. No differences in cardiac, gastrointestinal or renal involvement were found. No renal crisis occurred. Patient rated the global disease damage significantly higher in the anti-Scl-70+ group (48 vs 30 (VAS 0-100), p=0.031) and the Raynaud activity (45 vs 20 (VAS 0-100), p=0.022).Conclusions Anti-Scl70+ patients have distinct features compared to anti-Scl- patients, but interstitial lung disease is not more common at inclusion in the registry, despite the higher rate of CRP elevation. Pediatric organ involvement pattern clearly differs from adults.Abstract P.333 Table 1Comparison anti-Scl70 positive and negative patients at time of inclusion into the cohort",
  "authors": [
    {
      "affiliations": [
        "Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany"
      ],
      "name": "Ivan Foeldvari"
    },
    {
      "affiliations": [
        "German Rheumatism Research Center, Berlin, Germany"
      ],
      "name": "Jens Klotsche"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Kathryn Torok"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Ozgur Kasapcopur"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Brian Feldman"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Flavio Sztajnbok"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Maria Teresa Terreri"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Sindhu Johnson"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Jordi Anton"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Raju Khubchandani"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Valda Stanevicha"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Gülcan Özomay Baykal"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Dieneke Schonenberg-Meinema"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Ekaterina Alexeeva"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Maria Katsicas"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Sujata Sawhney"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Vanessa Smith"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Sima Abu Al-Saoud"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Eslam Al-Abadi"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Simone Appenzeller"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Stefanie Hajek"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Siri Opsahl Hetlevik"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Mikhail Kostik"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Suzanne Li"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Edoardo Marrani"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Anjali Patwardhan"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Natalia Vasquez-Canizares"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Natalia Cabrera"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Jorge M López Benítez"
    },
    {
      "affiliations": [
        "Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany"
      ],
      "name": "Nicola Helmus"
    }
  ],
  "title": "P.333 Is anti-Scl70 positivity associated with a more severe disease in juvenile systemic scleroderma",
  "uid": "f96d5db1-a258-5c08-9a93-8242b6b85991"
}