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Area of Science:

  • Pediatric Rheumatology
  • Neurology
  • Nephrology

Background:

  • Microscopic polyangiitis (MPA) is a rare small vessel vasculitis.
  • It is associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA).
  • Central nervous system (CNS) involvement in pediatric MPA is exceptionally rare.

Observation:

  • A 14-year-old girl presented with arthralgia and seizures.
  • She had leukocytoclastic vasculitis, interstitial lung disease with pulmonary hemorrhage, and pauci-immune glomerulonephritis.
  • High MPO-ANCA titers confirmed the diagnosis of MPA.

Findings:

  • Brain MRI revealed diffuse parenchymal involvement.
  • Lesions resolved within six months.
  • New microhemorrhage foci appeared, indicating ongoing vasculitic activity.

Implications:

  • This case expands the understanding of CNS manifestations in pediatric MPA.
  • It underscores the importance of neuroimaging in diagnosing and monitoring MPA in children.
  • The findings suggest a potential for recurrent CNS vascular events in pediatric MPA.