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Consensus-based recommendations for the management of juvenile systemic sclerosis
Ivan Foeldvari1, Roberta Culpo2, Francesca Sperotto2
1Hamburger Zentrum für Kinder- und Jugendrheumatologie, Schoen Clinic, Hamburg-Eilbek, Germany.
Rheumatology (Oxford, England)
|November 4, 2020
Summary
Juvenile systemic sclerosis (JSSc) lacks international treatment guidelines. This initiative offers expert-driven recommendations for JSSc assessment and treatment to improve pediatric patient outcomes.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Rare Childhood Conditions
Background:
- Juvenile systemic sclerosis (JSSc) is a rare pediatric autoimmune disease.
- Lack of international consensus on JSSc assessment and treatment hinders optimal patient care.
- Early recognition and management of internal organ involvement are crucial for long-term outcomes.
Purpose of the Study:
- To establish expert-driven recommendations for the assessment and treatment of JSSc.
- To improve the long-term outcomes for children diagnosed with JSSc.
- To standardize care for JSSc across Europe.
Main Methods:
- A European expert initiative (SHARE) synthesized best available evidence and expert opinion.
- Recommendations cover skin assessment and early detection of internal organ involvement.
- A proposed disease severity score for structured outcome assessment.
Main Results:
- Developed recommendations for comprehensive JSSc assessment, including internal organ monitoring.
- Proposed a stepwise treatment approach to standardize care.
- Introduced a disease severity score for outcome evaluation, requiring further validation.
Conclusions:
- The SHARE initiative provides crucial recommendations to unify JSSc care standards in Europe.
- Implementing these guidelines aims to reduce morbidity and mortality in pediatric patients with JSSc.
- Further validation of the proposed severity score in a larger patient cohort is recommended.
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