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Published on: October 14, 2021
[Juvenile dermatomyositis: clinical, immunologic, and therapeutic characteristics]
M A Faure-Fontenla1, R Rodríguez-Suárez, J J Sienra-Monge
1Departamento de Neumología y Alergía, Hospital Infantil de México Federico Gómez, México, D. F.
Insights
Childhood dermatomyositis is a rare but serious condition. Most children diagnosed with this autoimmune disease responded well to treatment, with many achieving remission.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Dermatomyositis
Context:
- Childhood dermatomyositis is a rare systemic autoimmune disease.
- Clinical presentation in children differs from adults due to increased vasculitis.
- Potential need for modified diagnostic criteria, disease course, and treatment in pediatric cases.
Purpose:
- To analyze the clinical experience and outcomes of childhood dermatomyositis.
- To evaluate the effectiveness of treatment and long-term prognosis in pediatric patients.
Summary:
- A 10-year study at Hospital Infantil de Mexico Federico Gomez included 21 children with dermatomyositis.
- All patients met the Bohan and Peters diagnostic criteria.
- 91% responded to treatment, with 52% achieving remission off-treatment and 37.7% improving with treatment. Mortality was 4.7% due to infections.
Impact:
- Highlights the distinct clinical features and treatment response in pediatric dermatomyositis.
- Suggests that established diagnostic criteria are applicable in children.
- Provides insights into disease management and prognosis for childhood dermatomyositis.
Abstract:
Although dermatomyositis is a rare disease in infancy, it involves several systems and tissues throughout the body causing death or serious disabilities at the musculoesquelethal level. The increased incidence of vasculitis and other characteristics in children make their clinical presentation different from the adult, which could modify the diagnostic criteria, course disease and treatment of the child. We analyze the clinical experience at Hospital Infantil de Mexico Federico Gomez with 21 children studied during a period of ten years. All patients fulfilled the Bohan and Peters diagnostic criteria and 91% of them responded to treatment. At the present time 52% are in remission without treatment, 37.7% improved with treatment, 4.5% with little response to treatment and 4.7% have died because of infections.
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