Hania S. Zayed, Tamer A. Gheita, Nevin Hammam, Manal Hassanien, Gehad Gamal Maghraby, Nermeen Samy, Nermeen N.Aziz, Abdelhfeez Moshrif, Yousra H. Abdel-Fattah, Amany R. El-Najjar, Enas A. Abdelaleem, Fatma A. Mohamed, Rania M. Gamal, Amany S. El-Bahnasawy, Khaled A. A. Abdelgalil, Dina F. El-Essawi, Reem El-Mallah, Thanaa F. Mansour, Eman F. Mohamed, Noha A. Aza
Cairo University. Assiut University. Ain Shams University. Al- Azhar University. Alexandria University. Zagazig University. Beni- Suef University. Minia University. Mansoura University. Aswan University. Egyptian Atomic Energy Authority. Tanta University.
Egypt
Scientific Reports
Sci Rep 2026; 16:
DOI: 10.1038/s41598-026-52795-y
Abstract
The aim of this work was to present the demographic and clinical features of children with juvenile systemic sclerosis (JSSc) compared to their older juvenile-onset (Jo-SSc) counterparts. The study included 12 JSSc children (< 16 years) and 54 Jo-SSc patients > 16 years (including adolescents and adults) recruited from 15 tertiary care centers across Egypt. Patients were classified as diffuse (dcSSc), limited cutaneous SSc (lcSSc) and overlap. The mean age at disease onset was 14 ± 2.5 years. Patients were 62 females and 4 males (F: M 15.5:1); 37 lcSSc, 26 dcSSc and 3 overlap. Disease features were generally comparable across five regions of Egypt. Pitting scars and hand puffiness were significantly more frequent in JSSc (58.3% and 41.7%) compared to Jo-SSc (16.7% and 14.8%) (p = 0.006 and p = 0.049, respectively) while pulmonary hypertension was more common in Jo-SSc (40.7% vs. 8.3%, p = 0.045). All males, currently with Jo-SSc, had gastrointestinal involvement versus n = 24 (38.7%) in females, p < 0.0001. To conclude, SSc in children and in those who grow into adulthood remains a rare disease. Pitting scars and hand puffiness were more frequent in children with JSSc while pulmonary hypertension became prominent as they grew. Children characteristics are special for each nation yet with similarities in some features.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes