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Anti-neutrophil cytoplasmic auto-antibodies-associated vasculitis with pulmonary and renal involvement
G Pintos-Morell1, A Roca-Comas, M A Naranjo
1Department of Paediatrics, University Hospital Germans Trias i Pujol, Badalona, Spain.
Abstract:
We present a 13-year-old boy with a rapidly progressive glomerulonephritis and pulmonary haemorrhage with perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) corresponding to anti-myeloperoxidase antibodies. The diagnosis of microscopic polyarteritis was made on the basis of the clinical features, the positivity of pANCA, and the histological finding of a pauci-immune crescentic glomerulonephritis. He responded excellently to corticosteroids and cyclophosphamide therapy and complete clinical remission persists 1 year after withdrawal of treatment. We emphasize the usefulness of ANCA antibody assays to establish a prompt diagnosis and adequate treatment in systemic vasculitis in children.
Insights
This study reports a pediatric case of microscopic polyarteritis, a rare vasculitis presenting with kidney and lung damage. Prompt diagnosis using anti-neutrophil cytoplasmic auto-antibody (ANCA) testing led to effective treatment and sustained remission.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Immunology
Background:
- Systemic vasculitis in children is rare but can cause severe organ damage.
- Microscopic polyarteritis (MPA) is a type of ANCA-associated vasculitis.
- Anti-neutrophil cytoplasmic auto-antibody (ANCA) assays are crucial for diagnosis.
Observation:
- A 13-year-old boy presented with rapidly progressive glomerulonephritis and pulmonary hemorrhage.
- Serological testing revealed perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) specific for anti-myeloperoxidase (MPO).
- Histopathology confirmed pauci-immune crescentic glomerulonephritis.
Findings:
- The patient was diagnosed with microscopic polyarteritis based on clinical, serological, and histological findings.
- Treatment with corticosteroids and cyclophosphamide resulted in excellent clinical response.
- Complete remission was maintained for 1 year after treatment cessation.
Implications:
- ANCA antibody assays are vital for the prompt diagnosis of childhood systemic vasculitis.
- Early diagnosis and aggressive immunosuppressive therapy can lead to favorable outcomes in pediatric MPA.
- This case highlights the importance of considering ANCA-associated vasculitis in children with unexplained glomerulonephritis and pulmonary hemorrhage.