Clinical Characteristics, Autoantibody Profiles, and Therapeutic Outcomes of Juvenile Systemic Sclerosis: An 11-Year Single-Center Experience from China - Report - MDSpire

Clinical Characteristics, Autoantibody Profiles, and Therapeutic Outcomes of Juvenile Systemic Sclerosis: An 11-Year Single-Center Experience from China

  • By

  • Wang, Yating

  • Tang, Wenjing

  • Zhang, Yu

  • Zhang, Zhi-Yong

  • An, Yunfei

  • Tang, Xuemei

  • April 29, 2026

  • 0 min

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Clinical Features, Autoantibody Profiles, and Treatment Outcomes in Pediatric Systemic Sclerosis

Overview

This study investigates juvenile systemic sclerosis (jSSc) in a cohort of 21 pediatric patients, highlighting significant clinical features, autoantibody profiles, and treatment outcomes over an 11-year period, with a median follow-up of 2.3 years. Key findings include the predominance of skin sclerosis and notable improvements in disease severity and skin involvement following treatment.

Background

Juvenile systemic sclerosis is a rare but severe autoimmune condition that can significantly impact growth and development in children. Understanding its clinical features and treatment responses is crucial for improving patient outcomes. This study provides valuable insights into the disease's manifestations and therapeutic approaches in a pediatric population.

Data Highlights

MeasurePre-treatmentPost-treatmentP-value
J4s (overall disease severity)5.94 ± 1.662.67 ± 2.220.003
mRSS (skin involvement)10.0 ± 9.217.42 ± 8.260.008

Key Findings

  • Skin sclerosis was the predominant symptom in all patients (100%).
  • Antinuclear antibody (ANA) was positive in 76.2% of cases.
  • Anti-single-stranded DNA (ssDNA) antibody was the most frequently observed specific antibody (23.8%).
  • Glucocorticoids and methotrexate were commonly used as initial treatments.
  • Significant reductions in overall disease severity and skin involvement were observed after treatment.
  • No fatalities or severe adverse events were reported during the follow-up period.

Clinical Implications

Discuss how findings can influence clinical practice, including the need for ongoing monitoring and adjustments in treatment strategies.

Conclusion

This study highlights the unique clinical features and treatment responses of jSSc in children, emphasizing the need for personalized therapeutic approaches to improve outcomes. Continued research is essential to refine treatment protocols and enhance patient care.

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  4. Clinical Rheumatology, 2021 -- Factors Influencing and Protective Elements of Achieving Low Disease Activity in Systemic Lupus Erythematosus: Insights from a Prospective Study in a Chinese Population
  5. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease - PMC, 2024
  6. Mycophenolate in Patients with Systemic Sclerosis–Associated Interstitial Lung Disease: A Systematic Review and Meta-Analysis | Annals of the American Thoracic Society
  7. Anti-RNA polymerase III antibodies in systemic sclerosis: prevalence and clinical associations from a systematic review and meta-analysis | Rheumatology
  8. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease - PMC
  9. Mycophenolate in Patients with Systemic Sclerosis–Associated Interstitial Lung Disease: A Systematic Review and Meta-Analysis | Annals of the American Thoracic Society | Oxford Academic
  10. Anti-RNA polymerase III antibodies in systemic sclerosis: prevalence and clinical associations from a systematic review and meta-analysis | Rheumatology | Oxford Academic

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