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Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study

Sarah-Louisa Mahi1, Siamak Bahram2, Jérôme Harambat3

  • 1Service de Pédiatrie 1, CHU University Hospital Hautepierre, 1, Avenue Molière, 67098, Strasbourg, France.

Pediatric Nephrology (Berlin, Germany)
|January 9, 2023
PubMed

Insights

Pediatric ANCA vasculitis shows increased incidence and frequent severe ENT involvement. Despite treatment changes, kidney outcomes remain poor, highlighting the need for further research into optimal therapies for children.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Nephrology

Background:

  • Pediatric ANCA-associated vasculitis (AAV) is rare with limited pediatric data.
  • Increasing incidence and evolving treatments like rituximab necessitate updated outcome analysis.
  • This study addresses the scarcity of information on AAV in children.

Purpose of the Study:

  • To characterize current pediatric AAV.
  • To analyze management strategies and outcomes.
  • To evaluate the impact of recent therapeutic trends.

Main Methods:

  • Retrospective study over 9 years.
  • Involved 46 children across 14 French centers.
  • Described clinical/laboratory features, treatments, and kidney outcomes.

Main Results:

  • P-ANCA positivity linked to higher relapse risk.
  • Frequent and severe ENT involvement noted in children compared to adults.
  • Poor kidney outcomes persisted (54.8% CKD at 1 year), with low mortality (6.5%).

Conclusions:

  • Long diagnosis times persist; P-ANCA is a significant relapse marker.
  • Treatment shifted towards rituximab, away from cyclophosphamide.
  • Kidney outcomes remain poor, underscoring the need for prospective studies on optimal therapies.
Abstract

Keywords:
ANCA-associated vasculitisKidney outcomePediatricRelapse

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