Granulomatosis With Polyangiitis in a Pediatric Male

Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has not been described in granulomatosis with polyangiitis (GPA) in a pediatric patient. We present a biopsy case that confirmed granulomatosis with polyangiitis with significant eosinophilia > 30% on the initial presentation. Etiologies that could account for eosinophilia were excluded during workup. The patient's presentation of pulmonary alveolar hemorrhage, conjunctivitis arthritis, high-titer cytoplasmic antineutrophil, PR3-ANCA antibodies, and cytoplasmic antibodies (cANCA) was consistent with a clinical picture of atypical GPA in a pediatric patient. This case presents a rare opportunity not only due to GPA’s low incidence in the pediatric population but due to the unusual nature of significant eosinophilia in GPA.