An updated overview of Juvenile systemic sclerosis in a French cohort

• DOI: 10.1186/s12969-024-01043-6
• Publication Date: 2025-02-08T14:47:17Z
• Authors: Léa Jacquel; Rouba Bechara; Joëlle Terzic; Anne-Cécile Rameau; Emmanuel Chatelus; Linda Rossi-Semerano; Isabelle Kone-Paut; Ulrich Meinzer; Irène Lemelle; Charlotte Rebelle; Diego Urbina; Pascal Pillet; Pauline Choquet; Jad El Maamari; Ariane Zaloszyc
• Keywords: RC925-935; Juvenile systemic sclerosis; Systemic; Autoimmune disease; Biological therapy; Diseases of the musculoskeletal system; Pediatrics; RJ1-570; Scleroderma; Research Article

Systemic sclerosis encompasses a range of disorders characterized by vascular and connective tissue abnormalities. Although rare in pediatrics, juvenile systemic (jSSc) is severe life-threatening condition that significantly impacts children's development. This study aimed to provide an overview JSSc France over the past decade. Patients with disease onset before age 16 were included following request for observations sent via email member practitioners SOFREMIP (French pediatric Rheumatology society). Our 18 patients from 8 different French centers. While our cohort exhibited balanced distribution between limited diffuse subsets disease, we observed higher prevalence subset children above 10. Skin induration was most reported symptom, while Raynaud's phenomenon present 61% at initial clinical evaluation. All tested positive antinuclear antibodies, anti-Scl70 being common specificity, even among cutaneous subsets. Interestingly, found high sensitivity ACR / EULAR criteria diagnosing jSSc 83% meeting these criteria, except 3 who presented overlap syndromes. Despite frequent use corticosteroids onset, no deaths or renal crises reported. Three received treatment biological agents, specifically Rituximab Tocilizumab. but requiring rapid, specialized, multidisciplinary care. Further studies are needed validate proper diagnosis including syndromes evaluate biotherapies children.