An updated overview of Juvenile systemic sclerosis in a French cohort
Abstract Background Systemic sclerosis encompasses a range of disorders characterized by vascular and connective tissue abnormalities. Although rare in pediatrics, juvenile systemic sclerosis (jSSc) is a severe and life-threatening condition that significantly impacts children’s development. This st...
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BMC
2025-02-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | https://doi.org/10.1186/s12969-024-01043-6 |
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| author | 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 SOFREMIP |
| author_facet | 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 SOFREMIP |
| author_sort | Léa Jacquel |
| collection | DOAJ |
| description | Abstract Background Systemic sclerosis encompasses a range of disorders characterized by vascular and connective tissue abnormalities. Although rare in pediatrics, juvenile systemic sclerosis (jSSc) is a severe and life-threatening condition that significantly impacts children’s development. This study aimed to provide an overview of JSSc in France over the past decade. Methods Patients with disease onset before the age of 16 were included following a request for observations sent via email to member practitioners of the SOFREMIP (French pediatric Rheumatology society). Results Our study included 18 patients from 8 different French centers. While our cohort exhibited a balanced distribution between limited and diffuse subsets of the disease, we observed a higher prevalence of the diffuse subset in children above the age of 10. Skin induration was the most reported symptom, while Raynaud’s phenomenon was present in 61% of the children at initial clinical evaluation. All children tested positive for antinuclear antibodies, with anti-Scl70 being the most common specificity, even among children with limited cutaneous subsets. Interestingly, we found a high sensitivity of the ACR / EULAR criteria for diagnosing jSSc in our cohort with 83% of patients meeting these criteria, except for 3 children who presented with overlap syndromes. Despite the frequent use of corticosteroids at the onset, no deaths or renal crises were reported. Three patients received treatment with biological agents, specifically Rituximab and Tocilizumab. Conclusion JSSc is a rare but severe disease requiring rapid, specialized, and multidisciplinary care. Further studies are needed to validate proper diagnosis criteria including overlap syndromes and evaluate the use of biotherapies in children. |
| format | Article |
| id | doaj-art-22e6f8e0a3fa46e9a4ea8bd95636e204 |
| institution | Kabale University |
| issn | 1546-0096 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj-art-22e6f8e0a3fa46e9a4ea8bd95636e2042025-02-09T12:17:02ZengBMCPediatric Rheumatology Online Journal1546-00962025-02-012311910.1186/s12969-024-01043-6An updated overview of Juvenile systemic sclerosis in a French cohortLéa Jacquel0Rouba Bechara1Joëlle Terzic2Anne-Cécile Rameau3Emmanuel Chatelus4Linda Rossi-Semerano5Isabelle Kone-Paut6Ulrich Meinzer7Irène Lemelle8Charlotte Rebelle9Diego Urbina10Pascal Pillet11Pauline Choquet12Jad El Maamari13Ariane Zaloszyc14SOFREMIPDepartment of Clinical Immunology and Internal Medicine, University Hospital of StrasbourgDepartment of Pediatrics, University Hospital of StrasbourgDepartment of Pediatrics, University Hospital of StrasbourgDepartment of Pediatrics, Groupe Hospitalier de la région de Mulhouse et Sud AlsaceDepartment of Rheumatology, University Hospital of StrasbourgDepartment of Paediatric Rheumatology, CEREMAIA, Hôpital de Bicêtre, APHPDepartment of Paediatric Rheumatology, CEREMAIA, Hôpital de Bicêtre, APHPDepartment of General Pediatrics, Hôpital Robert DebréDepartment of Pediatrics, Nancy University HospitalDepartment of Pediatrics, Hôpital Marseille Saint JosephDepartment of Pediatrics, University Hospital of MarseilleDepartment of Pediatrics, Pellegrin Hôpital des enfants, University Hospital of BordeauxDepartment of Pediatrics, Hospital of Annecy GenevoisDivision of Pediatric Hematology / Oncology, BC Children’s HospitalDepartment of Pediatrics, University Hospital of StrasbourgAbstract Background Systemic sclerosis encompasses a range of disorders characterized by vascular and connective tissue abnormalities. Although rare in pediatrics, juvenile systemic sclerosis (jSSc) is a severe and life-threatening condition that significantly impacts children’s development. This study aimed to provide an overview of JSSc in France over the past decade. Methods Patients with disease onset before the age of 16 were included following a request for observations sent via email to member practitioners of the SOFREMIP (French pediatric Rheumatology society). Results Our study included 18 patients from 8 different French centers. While our cohort exhibited a balanced distribution between limited and diffuse subsets of the disease, we observed a higher prevalence of the diffuse subset in children above the age of 10. Skin induration was the most reported symptom, while Raynaud’s phenomenon was present in 61% of the children at initial clinical evaluation. All children tested positive for antinuclear antibodies, with anti-Scl70 being the most common specificity, even among children with limited cutaneous subsets. Interestingly, we found a high sensitivity of the ACR / EULAR criteria for diagnosing jSSc in our cohort with 83% of patients meeting these criteria, except for 3 children who presented with overlap syndromes. Despite the frequent use of corticosteroids at the onset, no deaths or renal crises were reported. Three patients received treatment with biological agents, specifically Rituximab and Tocilizumab. Conclusion JSSc is a rare but severe disease requiring rapid, specialized, and multidisciplinary care. Further studies are needed to validate proper diagnosis criteria including overlap syndromes and evaluate the use of biotherapies in children.https://doi.org/10.1186/s12969-024-01043-6SclerodermaSystemicJuvenile systemic sclerosisAutoimmune diseaseBiological therapy |
| spellingShingle | 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 SOFREMIP An updated overview of Juvenile systemic sclerosis in a French cohort Pediatric Rheumatology Online Journal Scleroderma Systemic Juvenile systemic sclerosis Autoimmune disease Biological therapy |
| title | An updated overview of Juvenile systemic sclerosis in a French cohort |
| title_full | An updated overview of Juvenile systemic sclerosis in a French cohort |
| title_fullStr | An updated overview of Juvenile systemic sclerosis in a French cohort |
| title_full_unstemmed | An updated overview of Juvenile systemic sclerosis in a French cohort |
| title_short | An updated overview of Juvenile systemic sclerosis in a French cohort |
| title_sort | updated overview of juvenile systemic sclerosis in a french cohort |
| topic | Scleroderma Systemic Juvenile systemic sclerosis Autoimmune disease Biological therapy |
| url | https://doi.org/10.1186/s12969-024-01043-6 |
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