Diffuse pulmonary hemorrhage in children is rare and a potentially life threatening situation. We present one such case secondary to Perinuclear Antineutrophil Cytoplasmic Antibodies (pANCA) positive systemic vasculitis requiring Extra Corporeal Membrane Oxygenation (ECMO)
A sixteen-year old Caucasian boy presented to the emergency department with acute history of cough, hemoptysis and severe respiratory distress. In the past six months, he had episodes of cough and occasional hemoptysis; rhinorrhea and ankle swelling with erythematous skin rashes which were treated symptomatically. Urine examination revealed mild proteinuria and a renal ultrasound was reported as normal.Patient was emergently intubated endotracheally and mechanically ventilated with high settings with FiO2 of 1.0 and PEEP of 16. Chest x-ray revealed diffuse bilateral pulmonary infiltrates (Figure 1). Bronchial secretions were sent to test for unusual infections. Urine examination showed proteinuria and hematuria. Serologic investigations for systemic vasculitis were sent.Broad-spectrum anti-microbials and high dose methylprednisolone therapy were started. Patient rapidly deteriorated with progressive hypoxemia and pulmonary air-leaks. A brief trial of high frequency ventilation was ineffective. Patient was emergently placed on veno-venous ECMO. pANCA levels were positive the next day. Patient was started on a regimen of plasmapheresis, pulse methylprednisolone and cyclophosphamide. Patient showed dramatic improvement in 72 hours.He was weaned off ECMO on the sixth day and extubated the next day (Figure 2). Renal biopsy showed pauci-immune necrotizing crescentic glomerulonephritis.Patient was discharged home on maintenance steroid therapy.DISCUSSION: pANCA postive systemic vasculitis with diffuse alveolar haemorrhage and fulminant hypoxemic respiratory failure as a presenting feature is quite uncommon in children. Aggressive supportive care including ECMO can be life-saving (1) while the autoimmune vasculitis is being treated with pulse dose steroids, cyclophosphamide and plasmapheresis.
ECMO is an effective and safe supportive management strategy for fulminant acute hypoxemic respiratory failure secondary to pANCA positive systemic vasculitis even in the presence of pulmonary hemorrhage.
H.S. Agarwal, None.