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Non-specific aortoarteritis (NSAA) in children: a prospective observational study
Himanshu Gupta1, Navjyot Kaur1, Anita Saxena2
1Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
BMJ Paediatrics Open
|August 26, 2021
Summary
Non-specific aortoarteritis (NSAA) in children often presents atypically, with heart failure common. Early treatment improves outcomes, though long-term follow-up is needed.
Area of Science:
- Pediatric Rheumatology
- Cardiology
- Vascular Medicine
Background:
- Limited prospective data exists on the clinical profile, natural history, and outcomes of non-specific aortoarteritis (NSAA) in children.
- NSAA, a rare vasculitis, significantly impacts pediatric cardiovascular health.
Purpose of the Study:
- To evaluate the short-term and medium-term clinical outcomes of NSAA in pediatric patients.
- To characterize the presentation and disease course of NSAA in children.
Main Methods:
- A prospective observational study was conducted on 28 children (<15 years) diagnosed with NSAA at a tertiary care hospital in India.
- Data collected included clinical profile, symptoms, left ventricular ejection fraction (LVEF), vessel involvement, complications, and disease activity markers.
- Patients were followed for a mean of 13.5 months for disease activity and outcomes, receiving treatment per current guidelines.
Main Results:
- The cohort (mean age 10±2.9 years, 14 boys) showed 71% hypertension and 50% presented with acute decompensated heart failure (ADHF).
- 75% met modified Ishikawa criteria; 36% had active disease treated with immunosuppressants.
- 93% underwent interventions; LVEF and hypertension control improved, with no mid-term mortality, but 4 developed restenosis.
Conclusions:
- Pediatric NSAA presentation differs from adults, with ADHF and ventricular dysfunction being common.
- Effective immunosuppressive therapy and timely interventions are crucial for improving medium-term outcomes in pediatric NSAA.
- Further research is necessary to understand the long-term prognosis of NSAA in children.
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