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[Takayasu arteritis in pediatric patients]
María Martha Katsicas1, Luis Pompozi, Ricardo Russo
1Servicio de Inmunología y Reumatología, Hospital de Pediatría Prof. Juan P. Garrahan, Argentina. mmkatsi@yahoo.com.ar
Insights
Takayasu's arteritis, a rare pediatric vasculitis, presents diverse symptoms like claudication and hypertension. Early diagnosis and treatment are crucial for managing this potentially life-threatening large vessel inflammatory disease.
Area of Science:
- Pediatric rheumatology
- Vascular inflammation
- Autoimmune diseases
Context:
- Takayasu's arteritis is a significant cause of vasculitis in children, affecting major arteries like the aorta.
- It is the third most common vasculitis in the pediatric population.
- Understanding its pediatric manifestations is critical for timely intervention.
Purpose:
- To detail the clinical features, diagnostic findings, and treatment strategies for pediatric Takayasu's arteritis.
- To analyze a case series of children diagnosed with this condition.
- To highlight the diagnostic challenges and therapeutic approaches in pediatric patients.
Summary:
- Eleven pediatric patients (10 girls, median age 8) with Takayasu's arteritis were studied.
- Common symptoms included claudication, hypertension, and CNS involvement; elevated ESR, anemia, and thrombocytosis were noted.
- Vascular imaging revealed stenosis, dilatation, occlusion, and aneurysms, with outcomes ranging from remission to death.
Impact:
- Takayasu's arteritis can be life-threatening in children, necessitating high clinical suspicion.
- The study underscores the importance of recognizing varied symptoms for prompt diagnosis.
- Effective management involves steroids and immunosuppressants, though outcomes vary, emphasizing the need for ongoing research.
Abstract:
Takayasu's arteritis is a chronic inflammatory disease that primarily affects the large vessels, such as the aorta and its branches. It represents the third most frequent vasculitis during pediatric age. Our objective was to describe clinical and complementary exams features as well as treatment modalities of a case series of pediatric patients. We present 11 patients (10 girls) with median age at onset of 8 years (range: 2-15). The median diagnosis delay was 16 months (range: 2-96). Clinical presentations were lower limb claudication, arterial hypertension, CNS involvement, presence of murmurs, systemic symptoms, lymphadenopathy, chest pain, abdominal pain and arthritis. Laboratory tests showed: elevated ESR, anemia and trombocytosis. Vascular imaging studies exhibited stenosis, dilatation, occlussion and aneurysms. The outcome of the disease was persistent active condition (1 patient), relapse (4 patients), remission (3 patients), motor sequelae (1 patient) and death (2 patients). All patients were treated with steroids and immunosuppressants. Takayasu 's arteritis is a condition that can potentially be life-threatening. The diagnosis should be suspected in a variety of clinical manifestations during childhood.
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