作者: Abdurhman S. Al Arfaj , Mohammad Al Anazi , Najma Khalil , Akbar Ali Khan Pathan , Narsimha Reddy Parine
关键词: Necrotizing Vasculitis 、 Granulomatosis with polyangiitis 、 Churg-strauss syndrome 、 Eosinophilia 、 Medicine 、 ANCA-Associated Vasculitis 、 Asthma 、 Immunology 、 Eosinophilic
摘要: Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases almost three-fourths family members affected by asthma. explored genetic basis in this found that genes were mutated four siblings suggesting involvement susceptibility to EGPA.