Childhood ANCA Positive Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA) small and medium sized vasculitis. It is rare in childhood and its clinical presentation can be quite diverse. Objectives: to determine differences in clinical presentations, specific organ involvement and five factor score (FFS) between ANCA positive and ANCA negative cases. Methods: Descriptive statistical analysis was performed on Medline database up to June 2015 for childhood EGPA aged ? 18 years. Results: Sixty seven cases of childhood EGPA were identified with ANCA was measured in 35 cases (eight of them had positive results). In addition to the previous reported cases, we reported an eight years old female with severe asthma who presented with seizures and fingers’ gangrene with positive ANCA, radiological paranasal sinus abnormalities and bilateral pulmonary infiltrates. Central nervous system and ocular involvement were significantly higher among ANCA positive cases. Asthma, pulmonary infiltrates, paranasal sinuses, renal, gastrointestinal and musculoskeletal involvements were more frequent in ANCA positive cases. Peripheral neuropathy and cardiac involvement were less frequent in ANCA positive cases. Five factor score (FFS) ? 1 was more frequent in ANCA positive cases. Conclusion: Hypereosinophila in severe asthma should raise the suspicion of EGPA especially if associated with pulmonary infiltrates or unusual multisystem affection. ANCA measurement could be used as a prognostic factor for EGPA.