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Eosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients

 Chan-Bum Choi  ;  Yong-Beom Park  ;  Sang-Won Lee 
 Yonsei Medical Journal, Vol.60(8) : 705-712, 2019 
Journal Title
 Yonsei Medical Journal 
Issue Date
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Asian Continental Ancestry Group* ; Granulomatosis with Polyangiitis/classification ; Granulomatosis with Polyangiitis/pathology* ; Granulomatosis with Polyangiitis/therapy ; Granulomatosis with Polyangiitis/virology ; Hepatitis B virus/physiology ; Humans ; Prognosis ; Republic of Korea
Churg-Strauss syndrome ; Eosinophilic granulomatosis with polyangiitis ; classification ; clinical aspects ; treatment
Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Park, Yong Beom(박용범)
Lee, Sang Won(이상원) ORCID logo https://orcid.org/0000-0002-8038-3341
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