Hagemann Jan, Laudien Martin, Becker Sven, Cuevas Mandy, Klimek Felix, Kianfar Roya, Casper Ingrid, Klimek Ludger
Clinic for Ear, Nose and Throat Medicine, Mainz University Medical Center, Mainz.
Center for Rhinology and Allergology, Wiesbaden, Germany.
Allergol Select. 2024 Mar 21;8:18-25. doi: 10.5414/ALX02475E. eCollection 2024.
Eosinophilic granulomatosis with polyangiitis (EGPA) was formerly known as Churg-Strauss syndrome. The condition is characterized by disseminated necrotizing vasculitis with extravascular granulomas associated with hypereosinophilia. The vasculitides affect small vessels and are associated with antineutrophil cytoplasmic antibodies (ANCAs) detectable in the blood. Distinguishing between type 2-mediated chronic airway inflammation such as chronic rhinosinusitis with nasal polyps (CRSwNP) without vasculitis can be clinically challenging and should be considered.
Immunological background, diagnosis, and therapy of EGPA were identified through literature searches in Medline, PubMed, as well as national and international studies (ClinicalTrials.gov) and the Cochrane Library. Human studies published up to and including 10/2023 on the topic were considered.
In cases of deteriorating general health with previously known eosinophilic inflammation of the upper and lower airways, EGPA and its interdisciplinary investigation should be considered. Various types of eosinophilic inflammation and syndromes must be considered differentially.
Characterization of mucosal airway inflammation through biomarker determination is meaningful and occasionally makes the difference for targeted therapy.
嗜酸性肉芽肿性多血管炎（EGPA）以前被称为变应性肉芽肿性血管炎（CSS）。该病的特征是弥漫性坏死性血管炎伴血管外肉芽肿，伴有嗜酸性粒细胞增多。血管炎累及小血管，与血液中可检测到的抗中性粒细胞胞浆抗体（ANCA）相关。区分2型介导的慢性气道炎症，如无血管炎的慢性鼻-鼻窦炎伴鼻息肉（CRSwNP），在临床上具有挑战性，应予以考虑。
通过检索Medline、PubMed以及国内和国际研究（ClinicalTrials.gov）和Cochrane图书馆，确定EGPA的免疫背景、诊断和治疗方法。纳入截至2023年10月（含）发表的关于该主题的人体研究。
对于既往已知有上、下气道嗜酸性炎症且全身健康状况恶化的病例，应考虑EGPA及其多学科检查。必须对各种类型的嗜酸性炎症和综合征进行鉴别。
通过生物标志物测定来表征黏膜气道炎症是有意义的，有时对靶向治疗至关重要。
