Abstract

RODRIGUEZ-ARMENDARIZ, Rubén et al. Eosinophilic granulomatosis with polyangiitis: a case report and literature review. Neumol. cir. torax [online]. 2017, vol.76, n.1, pp.36-43. ISSN 0028-3746.

Eosinophilic granulomatosis with poliangitis (EGPA), formerly called Churg-Strauss syndrome, is a rare disorder of unknown etiology characterized by necrotizing vasculitis that affects small vessels of medium caliber. Asthma, allergic rhinitis and peripheral blood eosinophilia are present in most patients. The EGPA in childhood is a rare clinical condition. Although EGPA is a vasculitis with neutrophil cytoplasmic antibodies related (ANCA) disorder in children, in less than 40% of all vasculitis cases are found. Early detection is important because a delay in diagnosis can lead to organ damage of varying degrees, some of them fatal. EGPA starting in childhood, represents less than 2% of cases of vasculitis in pediatric age and only 50 cases of SCS in children (< 18 years old) had been published before 2013. We report the case of a 13 year old boy with difficult to control asthma and impaired general condition, which was finally diagnosed with EGPA, ANCA-negative.

Keywords : Eosinophilic granulomatosis with polyangiitis; EGPA; asthma; Churg Strauss; vasculitis.

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