Eosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients

Abstract

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.

Eosinophilic Granulomatosis With Polyangiitis With Extensive Cutaneous Involvement

“…EGPA is a heterogeneous disease that presents with allergic and vasculitic features. Classically, three clinical stages are described: allergic stage, eosinophilic stage and vasculitic stage [4][5][6]. Our patient had a history of adult-onset asthma, chronic rhinitis and nasal polyposis, which correspond to the first stage.…”

“…Then, there is an eosinophilic stage with tissue and peripheral eosinophilia. At last, the vasculitic stage is marked by necrotizing inflammation of medium and small vessels, extravascular granulomatosis, and end-organ damage [4][5][6].…”

“…Clinical differences associated with ANCA status have been described. Mononeuritis multiplex, glomerular nephritis and alveolar hemorrhage are more frequent in ANCA-positive patients, whereas heart involvement is more frequent in ANCA-negative patients [ 1 , 3 , 5 - 6 ].…”

Serum Clusterin Level Could Reflect the Current Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

“…EGPA has different pathogenesis and treatment strategies compared to MPA and GPA. For this reason, 21 large-scale clinical studies are sometimes conducted with only MPA and GPA patients. 9 20 22 Therefore, we included only 29 MPA patients and 17 GPA patients and analysed them again.…”

Епидемиология На Еозинофилната Грануломатоза С Полиангиит

“…За поставяне на диагноза EGPA пациентите трябва да отговарят на определени критерии, фигуриращи в поне една от препоръките на: Американски Колегиум по ревматология (American College of Rheumatology, ACR) за грануломатозен полиангиит (1990), консенсусната конференция от Chapel Hill за номенклатура на васкулитите (Chapel Hill Consensus Conference, CHCC) (1994, 2012) и алгоритъма на Европейската агенция по лекарствата (European Medical Agency, EMA) (2007) [7][8][9][10]. През 2017 г. пред ACR и Европейската Лига срещу Ревматизма (European League Against Rheumatism, EULAR) са предложени нови класификационни критерии за първичните системни васкулити, предоставящи 85% сензитивност и 99% специфичност за диагностициране на EGPA [7,11,12].…”