Clinical Characteristics of Children with Juvenile Systemic Sclerosis
Author Information
Author(s): Russo Ricardo AG, Katsicas María M
Primary Institution: Hospital de Pediatría Prof. Dr. Juan P. Garrahan
Hypothesis
The study aims to describe and analyze the clinical and laboratory characteristics of children with Juvenile Systemic Sclerosis (JSS).
Conclusion
Raynaud's phenomenon is the initial symptom of JSS, and capillaroscopy is crucial for diagnosis, as skin and vascular manifestations are the most common features.
Supporting Evidence
- Raynaud's phenomenon was the first symptom in 14 out of 23 patients.
- Proximal sclerosis was observed in all patients.
- ANA were present in 74% of the children.
- Respiratory involvement occurred in two thirds of the patients.
- Dysphagia was the most common gastrointestinal symptom.
Takeaway
Juvenile systemic sclerosis is a rare disease that often starts with a symptom called Raynaud's phenomenon, and doctors use a special test called capillaroscopy to help diagnose it.
Methodology
Clinical charts of children diagnosed with JSS were reviewed from a tertiary referral center between 1995 and 2005.
Limitations
The study is limited by its small sample size and the retrospective nature of the data collection.
Participant Demographics
The cohort consisted of 23 patients, predominantly girls (21), with ages ranging from 1 to 14 years at the onset of symptoms.
Digital Object Identifier (DOI)
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