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[Sharp syndrome in an infant: a case report]
Pediatrie
|January 1, 1992
Insights
Sharp syndrome, a rare autoimmune disorder, was diagnosed late in an 11-year-old boy. Treatment with corticosteroids and antimalarial drugs led to satisfactory long-term outcomes.
Area of Science:
- Rheumatology
- Pediatric Autoimmunity
- Systemic Autoimmune Diseases
Background:
- Sharp syndrome, also known as mixed connective tissue disease (MCTD), is a rare autoimmune disorder.
- Late diagnosis in pediatric cases can complicate management and prognosis.
Observation:
- An 11-year-old boy with late-diagnosed Sharp syndrome experienced disease progression.
- He presented with myopericardic aggravation, glomerular disease, and seizures with delirium despite initial corticosteroid therapy.
Findings:
- High-dose steroid therapy initially improved symptoms.
- Early switch to synthetic antimalarial drugs resulted in sustained satisfactory outcomes over six years.
Implications:
- This case highlights the importance of timely diagnosis and aggressive management in pediatric Sharp syndrome.
- Synthetic antimalarial drugs may be a valuable therapeutic option for managing refractory or advanced cases, offering long-term benefits.
Abstract:
We report on a case of Sharp syndrome which was diagnosed late in on 11-year old boy. After 5 years of corticosteroid treatment, he presented with myopericardic aggravation, glomerular disease, and seizures with delirium, which responded to high-dose steroid therapy with early switch using synthetic antimalarial drugs. Six years later the results are still satisfactory.