Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with pulmonary hemorrhage is rare in childhood. Standard treatment includes corticosteroids and cyclophosphamide (CYC), which is associated with a high level of toxicity. We report a white female with ANCA positive pulmonary hemorrhage who was treated with cyclophosphamide (CYC) and rituximab (RTX) combination therapy.
Highlights
associated vasculitis (AAV) with pulmonary hemorrhage is rare in childhood
Untreated AAV with pulmonary hemorrhage is associated with a 2 year mortality rate in excess of 90% [1]
We present a case of recurrent pulmonary hemorrhage associated with antineutrophil antibody (ANCA)-associated vasculitis (AAV) in childhood
Summary
AAV with pulmonary hemorrhage is rare in childhood. Untreated AAV with pulmonary hemorrhage is associated with a 2 year mortality rate in excess of 90% [1]. Almost 3 years later, at 10 years of age, a chest radiograph showed worsening diffuse airspace opacities, pulmonary interstitial emphysema, and a pneumo-mediastinum following Streptococcal pharyngitis She again required admission and mechanical ventilation for pulmonary hemorrhage and respiratory failure. She was discharged after nineteen days on prednisolone of 1 mg/kg daily Because she had recurrent episodes of life threatening pulmonary hemorrhage despite 3 years of chronic steroid administration, we elected to treat her aggressively for AAV at 10 years of age. After appropriate discussion of the risks and benefits, consent was obtained and she was hospitalized for CYC and RTX treatment (750 mg/m2 of CYC and 600 mg/m2 of RTX with a maximum of 1000 mg) Following this therapy she was discharged on prednisolone of 30 mg daily which was tapered off gradually over five months. There have been no further episodes of pulmonary hemorrhage, and she remains well without corticosteroid therapy two years following her last infusion
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