臨床小児科: オープンアクセス
オープンアクセス

概要

Eosinophilic Granulomatosis polyangiitis (EGPA) in Pediatrics Population, Rare Disease with a Challenging Diagnosis: Case Report

Tarek Husien*

Eosinophilic Granulomatosis polyangiitis (EGPA) is a rare ANCA-associated multisystem vasculitides that affects mainly adult population. Occurrence in pediatrics population is extremely rare with only few cases been reported in adolescent patients. Eosinophilic granulomatosis with polyangiitis (EGPA)—or, as it was traditionally termed, Churg-Strauss syndrome—is a rare systemic necrotizing vasculitis that affects small-to-medium-sized vessels and is associated with severe asthma and blood and tissue eosinophilia. Like granulomatosis with polyangiitis (Wegener granulomatosis), and the microscopic form of periarteritis, EGPA is an antineutrophil cytoplasmic antibody (ANCA)–associated vasculitide