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Nodular pulmonary vasculitis in a twelve-year-old boy
T Borchers1, O Dirsch, J Schaper
1Children's Hospital, University of Essen, Essen, Germany. tanja.borchers@uni-essen.de
Pediatric Pulmonology
|January 20, 2004
Summary
A pediatric case of primary pulmonary vasculitis, negative for c-ANCA, was successfully treated with surgery. This rare condition, presenting as a solitary pulmonary nodule, highlights a unique childhood vasculitis presentation.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Vascular Pathology
Background:
- Childhood vasculitis is rare, often presenting with systemic symptoms.
- Primary pulmonary vasculitis without systemic involvement is exceptionally uncommon in pediatric patients.
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis typically involves the c-ANCA antibody, particularly in Wegener's granulomatosis.
Observation:
- A 12-year-old boy presented with shoulder pain, sweating, and fatigue.
- Diagnostic imaging revealed a solitary pulmonary nodule.
- Elevated p-ANCA (antineutrophil cytoplasmic antibodies directed against myeloperoxidase) was noted, but c-ANCA (antineutrophil cytoplasmic antibodies directed against affinity purified proteinase 3) was negative.
Findings:
- Surgical resection and pathological examination confirmed localized granulomatous vasculitis.
- Post-surgery, p-ANCA levels normalized within 6 months.
- The patient experienced a favorable 2-year follow-up without recurrence.
Implications:
- This case represents the first reported instance of c-ANCA negative primary pulmonary vasculitis in a child.
- It expands the spectrum of ANCA-associated vasculitis presentations in pediatric populations.
- Highlights the importance of considering localized vasculitis in children presenting with pulmonary nodules and relevant autoantibodies.