{
  "abstract": "Introduction Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of 3 in 1 000 000 children. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects data multinationally, including clinical manifestations, auto-antibodies profile and patient and physician reported outcomes. Disease presentation can be different in the prepubertal age in lot of diseases.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 reviewed data from patients in the jSScC at the time of inclusion in the registry until 15th of December 2024.Results We studied 260 patients, 70% Caucasian, 80% female, in 41% (n=107) the age of onset was under 9 years (jSSc<9). The patients with jSSc<9 had a non-significant higher number of diffuse subset patients (74% versus 65%). The median disease duration was 4.2 in the jSSc<9 versus 1.9 years in jSSc>9. The female to male ratio was higher in jSSc <9 (4.6:1 versus 3.6:1). Regarding antibody pattern the only difference found between jSSc<9 and jSSc>9 was a significantly higher number of patients with anti-PM-Scl positivity (24% versus 10%; p=0.048). The jSSc<9 group had a more frequent history of ulceration (61% versus 46%, p value nonsignificant (NS)) and higher number of calcinosis (25% versus 9%, p=0.021). The number of patients with pulmonary hypertension assessed by ultrasound was lower in the jSSc<9 group (3% versus 8%, p value NS). Renal involvement, mostly proteinuria, was lower in the jSSc<9 with 1% versus 4%, p value NS. Renal crisis was not reported. Number of patients with decreased Body Mass Index z score <2 was higher in the jSSc<9 group (19% versus 12%, p value NS). Tendon friction rub was less common in the jSSc<9 (2% versus 9%, p value NS). There was no difference in the assessed patient and physician related outcomes. The mean CHAQ was 0.5 in both groups.Conclusions In the currently in this largest jSSc cohort of the world patients in the jSSc<9 group seems for have slightly more severe disease. It is remarkable the significant higher number of patients with anti-PM-Scl antibodies in the jSSc<9 group.Abstract P.331 Table 1",
  "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": "Raju Khubchandani"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Jordi Anton"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Gülcan Özomay Baykal"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Sindhu Johnson"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Dieneke Schonenberg-Meinema"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Valda Stanevicha"
    },
    {
      "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": "Natalia Cabrera"
    },
    {
      "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": "Suzanne Li"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Edoardo Marrani"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Clare Pain"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Anjali Patwardhan"
    },
    {
      "affiliations": [
        "jSSc Collaborative Group, Hamburg, Germany"
      ],
      "name": "Berntson Lillemor"
    },
    {
      "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.331 Age of onset effect on the clinical manifestations of patients with juvenile systemic sclerosis",
  "uid": "0ad10907-3b40-5f88-812c-b10c1d7511cc"
}