Abstract
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.
Highlights
Granulomatosis with polyangiitis (GPA) is a multisystem necrotizing granulomatous vasculitis of the small to medium-sized vessels that usually presents between the ages of 40 and 60 years [1,2,3]
We present a biopsy case that confirmed granulomatosis with polyangiitis with significant eosinophilia > 30% on the initial presentation
Notable features seen in our patient that coincide with other atypical granulomatosis with polyangiitis (GPA) cases are the presence of significant pulmonary alveolar hemorrhage, conjunctivitis arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibodies, and proteinase-3 antibodies
Summary
Granulomatosis with polyangiitis (GPA) is a multisystem necrotizing granulomatous vasculitis of the small to medium-sized vessels that usually presents between the ages of 40 and 60 years [1,2,3]. A pediatric case of GPA is a rare opportunity presenting with a pediatric occurrence rate of 1:1,000,000; such a presentation, in addition to the presence of significant eosinophilia, has yet to be described in the literature [3]. A 17-year-old African American male presented to the emergency department with arthralgias, fever, nausea, and vomiting that was worsening for the last four days. He had been seen at a rural ED twice in the last two weeks for headaches and arthralgias, where he was diagnosed with viral upper respiratory tract infection and tested COVID-19 negative via polymerase chain reaction (PCR).
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