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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Juvenile systemic sclerosis
Ivan Foeldvari1, Clare E Pain2
1Hamburg Centre for Pediatric and Adolescence Rheumatology, Centre for Treatment of Scleroderma and Uveitis in Childhood and Adolescence, Teaching Unit of the Asklepios Campus of the Semmelweis Medical School, Asklepius Campus, Hamubrg, Germany; An der Schön Klinik Hamburg Eilbek, Dehnhaide 120, 22081, Hamburg, Germany.
Best Practice & Research. Clinical Rheumatology
|February 4, 2026
Summary
Juvenile systemic sclerosis (jSSc) is a rare autoimmune condition affecting children, differing significantly from adult forms. This review details its unique features, assessment, and management strategies for better pediatric care.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Systemic Sclerosis
Background:
- Juvenile systemic sclerosis (jSSc) is a rare autoimmune disease affecting 4-10% of systemic sclerosis cases.
- jSSc presents unique phenotypes compared to adult-onset disease, including more diffuse skin involvement and distinct autoantibody profiles.
- Limited evidence exists due to rarity and lack of pediatric trials, necessitating specialized guidance.
Purpose of the Study:
- To review the epidemiology, classification, clinical features, and outcomes of jSSc.
- To highlight key differences between juvenile and adult systemic sclerosis.
- To provide a structured approach for assessment and management of jSSc.
Main Methods:
- Literature review focusing on epidemiology, classification, and clinical features of jSSc.
- Comparison of jSSc with adult-onset systemic sclerosis.
- Synthesis of paediatric consensus recommendations and extrapolated adult data for management strategies.
Main Results:
- jSSc exhibits distinct clinical features, autoantibody profiles, and outcomes compared to adult SSc.
- A structured, multisystem evaluation approach is crucial for comprehensive assessment.
- Management requires specialist pediatric rheumatology care within multidisciplinary teams.
Conclusions:
- jSSc requires tailored diagnostic and management strategies distinct from adult SSc.
- Multisystem evaluation and multidisciplinary care are essential for optimal patient outcomes.
- Further research and pediatric trials are needed to advance treatment options and address psychosocial impacts.
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