{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T10:49:19Z","timestamp":1785408559264,"version":"3.56.0"},"reference-count":119,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T00:00:00Z","timestamp":1780531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82271815"],"award-info":[{"award-number":["82271815"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"award":["82271815"],"award-info":[{"award-number":["82271815"]}],"id":[{"id":"https:\/\/ror.org\/01h0zpd94","id-type":"ROR","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous autoimmune disease involving multiple organs and systems, characterized by asthma, eosinophilia, and granulomatous or vasculitic involvement of several organs. Most of the initial symptoms include rhinitis, sinusitis, and asthma. The primary consultation departments are typically allergy, otolaryngology, and respiratory. EGPA lacks effective biomarkers for early diagnosis, and most patients have experienced serious multiple organ damage at the time of diagnosis, which makes it more difficult to treat. Therefore, as an allergist, improving the understanding of EGPA, especially the early identification of EGPA in the rhinitis, asthma, or sinusitis stage, will effectively shorten the overall course of EGPA and reduce the mortality and disability rates. Based on this, the author compiled a diagnosis and treatment pathway to outline the clinical manifestations; examination, diagnosis, and treatment pathways; differential diagnosis; disease management; and other aspects of EGPA in the allergic department, aiming to promote early detection, identification, and diagnosis by allergists.<\/jats:p>","DOI":"10.3390\/diagnostics16111734","type":"journal-article","created":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T11:52:30Z","timestamp":1780573950000},"page":"1734","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Diagnosis and Treatment Pathway for Eosinophilic Granulomatosis with Polyangiitis in the Allergy Department"],"prefix":"10.3390","volume":"16","author":[{"given":"Juan","family":"Liu","sequence":"first","affiliation":[{"name":"Beijing Key Laboratory of Precision Medicine for Diagnosis and Treatment of Allergic Diseases, Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China"},{"name":"National Clinical Research Center for Dermatologic and Immunologic Disease (NCRC-DID), Beijing 100730, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingyang","family":"Xu","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Precision Medicine for Diagnosis and Treatment of Allergic Diseases, Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China"},{"name":"National Clinical Research Center for Dermatologic and Immunologic Disease (NCRC-DID), Beijing 100730, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7539-6650","authenticated-orcid":false,"given":"Yuxiang","family":"Zhi","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Precision Medicine for Diagnosis and Treatment of Allergic Diseases, Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China"},{"name":"National Clinical Research Center for Dermatologic and Immunologic Disease (NCRC-DID), Beijing 100730, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"5120","DOI":"10.1038\/s41467-019-12515-9","article-title":"Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status","volume":"10","author":"Lyons","year":"2019","journal-title":"Nat. Commun."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1694","DOI":"10.1002\/art.41722","article-title":"Occupational Exposures and Smoking in Eosinophilic Granulomatosis with Polyangiitis: A Case\u2013Control Study","volume":"73","author":"Maritati","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1038\/s41584-023-00958-w","article-title":"Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis","volume":"19","author":"Emmi","year":"2023","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1002\/art.41986","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis","volume":"74","author":"Robson","year":"2022","journal-title":"Arthritis Rheumatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.jaip.2024.10.013","article-title":"Characteristics of Severe Asthma Clinic Patients With Eosinophilic Granulomatosis With Polyangiitis","volume":"13","author":"Puan","year":"2024","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2926","DOI":"10.1002\/art.21250","article-title":"Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome","volume":"52","author":"Sinico","year":"2005","journal-title":"Arthritis Rheum."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"00430","DOI":"10.1183\/23120541.00430-2023","article-title":"Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK","volume":"10","author":"Hwee","year":"2024","journal-title":"ERJ Open Res."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"100437","DOI":"10.1016\/j.jacig.2025.100437","article-title":"Eosinophilic granulomatosis with polyangiitis: Patient profiles from a large US allergy practice","volume":"4","author":"Wechsler","year":"2025","journal-title":"J. Allergy Clin. Immunol. Glob."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"3369","DOI":"10.1007\/s12325-025-03197-5","article-title":"Reality of Patient-Reported Symptoms in 200 Patients with Eosinophilic Granulomatosis with Polyangiitis: A Cross-Sectional Survey (The KUNPU Study)","volume":"42","author":"Amano","year":"2025","journal-title":"Adv. Ther."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"331","DOI":"10.5114\/reum\/211785","article-title":"Dual pathogenesis and treatment approaches for eosinophilic granulomatosis with polyangiitis: A comprehensive review","volume":"63","author":"Sebastian","year":"2025","journal-title":"Rheumatology"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Fagni, F., Bello, F., and Emmi, G. (2021). Eosinophilic Granulomatosis with Polyangiitis: Dissecting the Pathophysiology. Front. Med., 8.","DOI":"10.3389\/fmed.2021.627776"},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Fijolek, J., and Radzikowska, E. (2023). Eosinophilic granulomatosis with polyangiitis\u2014Advances in pathogenesis, diagnosis, and treatment. Front. Med., 10.","DOI":"10.3389\/fmed.2023.1145257"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"iii84","DOI":"10.1093\/rheumatology\/kez570","article-title":"Eosinophilic granulomatosis with polyangiitis: Understanding the disease and its management","volume":"59","author":"Trivioli","year":"2020","journal-title":"Rheumatology"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"L\u00f3pez Paraja, M., Starita Fajardo, G., Donate Velasco, I., Lucena L\u00f3pez, D., Iranzo Alcolea, M.P., Lirola S\u00e1nchez, F.J., Rodriguez, M.P., Garc\u00eda, A.G., and Espinosa, L.M. (2025). Molecular Pathogenesis and Targeted Therapies in Eosinophilic Granulomatosis with Polyangiitis: An Updated Review. Int. J. Mol. Sci., 26.","DOI":"10.3390\/ijms262211141"},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Dejaco, C., Oppl, B., Monach, P., Cuthbertson, D., Carette, S., Hoffman, G., Khalidi, N., Koening, C., Langford, C., and McKinnon-Maksimowicz, K. (2015). Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss). PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0121737"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1002\/1529-0131(200102)44:2<469::AID-ANR66>3.0.CO;2-0","article-title":"Elevated interleukin-4 and interleukin-13 production by T cell lines from patients with Churg-Strauss syndrome","volume":"44","author":"Kiene","year":"2001","journal-title":"Arthritis Rheum."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1093\/rheumatology\/kes171","article-title":"Increased production of IL-5 and dominant Th2-type response in airways of Churg\u2013Strauss syndrome patients","volume":"51","author":"Jakiela","year":"2012","journal-title":"Rheumatology"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Berti, A., and Pagnoux, C. (2025). Interleukin-5, Eosinophils, and Interleukin-5 Pathway Inhibitors in Eosinophilic Granulomatosis with Polyangiitis. Arthritis Rheumatol., Online ahead of print.","DOI":"10.1002\/art.43409"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1056\/NEJMoa1702079","article-title":"Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis","volume":"376","author":"Wechsler","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_20","first-page":"S23","article-title":"Both Th2 and Th17 responses are involved in the pathogenesis of Churg-Strauss syndrome","volume":"29","author":"Jakiela","year":"2011","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_21","first-page":"24","article-title":"Role of thymic stromal lymphopoietin in allergy and beyond","volume":"23","author":"Leonard","year":"2022","journal-title":"Nat. Rev. Immunol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"103219","DOI":"10.1016\/j.autrev.2022.103219","article-title":"Eosinophilic granulomatosis with polyangiitis: A review","volume":"22","author":"White","year":"2022","journal-title":"Autoimmun. Rev."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Matucci, A., Vivarelli, E., Perlato, M., Mecheri, V., Accinno, M., Cosmi, L., Parronchi, P., Rossi, O., and Vultaggio, A. (2023). EGPA Phenotyping: Not Only ANCA, but Also Eosinophils. Biomedicines, 11.","DOI":"10.3390\/biomedicines11030776"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1067\/mjd.2002.124600","article-title":"Evidence for pathogenic involvementof eosinophils and neutrophilsin Churg-Strauss syndrome","volume":"47","author":"Drage","year":"2002","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1136\/hrt.81.6.669","article-title":"Eosinophilic myocarditis associated with dense deposits of eosinophil cationic protein (ECP) in endomyocardium with high serum ECP","volume":"81","author":"Arima","year":"1999","journal-title":"Heart"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.alit.2022.02.009","article-title":"In vivo visualization of eosinophil secretion in eosinophilic granulomatosis with polyangiitis: An ultrastructural study","volume":"71","author":"Koike","year":"2022","journal-title":"Allergol. Int."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/s12959-015-0047-z","article-title":"Thrombosis in vasculitis: From pathogenesis to treatment","volume":"13","author":"Emmi","year":"2015","journal-title":"Thromb. J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"4523","DOI":"10.1182\/blood-2010-02-267542","article-title":"Interleukin-25: A cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome","volume":"116","author":"Terrier","year":"2010","journal-title":"Blood"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2662","DOI":"10.1111\/all.16303","article-title":"Interleukin-5 as a pleiotropic cytokine orchestrating airway type 2 inflammation: Effects on and beyond eosinophils","volume":"79","author":"Buchheit","year":"2024","journal-title":"Allergy"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1683","DOI":"10.1002\/art.41727","article-title":"Eosinophil ETosis\u2013Mediated Release of Galectin-10 in Eosinophilic Granulomatosis with Polyangiitis","volume":"73","author":"Fukuchi","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Piga, M.A., Fraticelli, P., Antonicelli, L., Garritani, M.S., Ghirelli, G., Martini, M., Di Vincenzo, A., Danieli, M.G., Moroncini, G., and Bil\u00f2, M.B. (2023). ANCA-negative EGPA: Only eosinophils without vasculitis? Insights from anti-T2 biologics. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1325299"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"180","DOI":"10.55563\/clinexprheumatol\/rrjt9m","article-title":"A new focus on thyrosine kinases inhibitors in eosinophilic granulomatosis with polyangiitis","volume":"39","author":"Triggianese","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"102737","DOI":"10.1016\/j.autrev.2020.102737","article-title":"Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis: A systematic literature review","volume":"20","author":"Akiyama","year":"2021","journal-title":"Autoimmun. Rev."},{"key":"ref_34","first-page":"e501","article-title":"Elevated Serum IgG4 Was Found in Eosinophilic Granulomatosis with Polyangiitis","volume":"27","author":"Wu","year":"2020","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e002086","DOI":"10.1136\/rmdopen-2021-002086","article-title":"Eosinophilic granulomatosis with polyangiitis exhibits T cell activation and IgG4 immune response in the tissue; comparison with IgG4-related disease","volume":"8","author":"Kubo","year":"2022","journal-title":"RMD Open"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1053\/j.ajkd.2005.01.001","article-title":"Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs","volume":"45","author":"Schlieben","year":"2005","journal-title":"Am. J. Kidney Dis."},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Kronbichler, A., Lee, K.H., Denicol\u00f2, S., Choi, D., Lee, H., Ahn, D., Kim, K.H., Lee, J.H., Kim, H.T., and Hwang, M. (2020). Immunopathogenesis of ANCA-Associated Vasculitis. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197319"},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Paroli, M., Gioia, C., and Accapezzato, D. (2023). New Insights into Pathogenesis and Treatment of ANCA-Associated Vasculitis: Autoantibodies and Beyond. Antibodies, 12.","DOI":"10.3390\/antib12010025"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.7150\/ijms.53728","article-title":"Neutrophil Extracellular Traps (NETs) and Vasculitis","volume":"18","author":"Arneth","year":"2021","journal-title":"Int. J. Med. Sci."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"3007","DOI":"10.1182\/blood-2012-03-416156","article-title":"Neutrophil extracellular traps mediate transfer of cytoplasmic neutrophil antigens to myeloid dendritic cells toward ANCA induction and associated autoimmunity","volume":"120","author":"Sangaletti","year":"2012","journal-title":"Blood"},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"Hashimoto, T., Ueki, S., Kamide, Y., Miyabe, Y., Fukuchi, M., Yokoyama, Y., Furukawa, T., Azuma, N., Oka, N., and Takeuchi, H. (2021). Increased Circulating Cell-Free DNA in Eosinophilic Granulomatosis with Polyangiitis: Implications for Eosinophil Extracellular Traps and Immunothrombosis. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.801897"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1002\/art.23209","article-title":"Associations of Churg-Strauss syndrome with the HLA\u2013DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody\u2013associated vasculitides: Comment on the article by Vaglio et al","volume":"58","author":"Wieczorek","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"3159","DOI":"10.1002\/art.22834","article-title":"HLA\u2013DRB4 as a genetic risk factor for Churg-Strauss syndrome","volume":"56","author":"Vaglio","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1839","DOI":"10.1002\/art.23496","article-title":"Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody\u2013negative Churg-Strauss syndrome, but not with Wegener\u2019s granulomatosis","volume":"58","author":"Wieczorek","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1016\/S0003-4967(24)43399-2","article-title":"Systemic small sized vessel vasculitis after massive antigen inhalation","volume":"60","author":"Mouthon","year":"2001","journal-title":"Ann. Rheum. Dis."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1093\/omcr\/omy126","article-title":"Chronic pulmonary aspergillosis may cause eosinophilic granulomatosis with polyangiitis via allergic bronchopulmonary aspergillosis","volume":"2019","author":"Harada","year":"2019","journal-title":"Oxf. Med. Case Rep."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.pulmoe.2021.12.004","article-title":"Concomitant allergic bronchopulmonary aspergillosis and eosinophilic granulomatosis with polyangiitis after Aspergillus niger infection","volume":"28","author":"Coutinho","year":"2022","journal-title":"Pulmonology"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"e382","DOI":"10.3346\/jkms.2023.38.e382","article-title":"Eosinophilic Granulomatosis with Polyangiitis Following COVID-19 Vaccination: A Case Report","volume":"38","author":"Hwang","year":"2023","journal-title":"J. Korean Med. Sci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1378\/chest.117.3.708","article-title":"Churg-Strauss Syndrome in Patients Receiving Montelukast as Treatment for Asthma","volume":"117","author":"Wechsler","year":"2000","journal-title":"Chest"},{"key":"ref_50","first-page":"214","article-title":"Omalizumab in eosinophilic granulomatosis with polyangiitis: Friend or foe? A systematic literature review","volume":"38","author":"Basta","year":"2020","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"755","DOI":"10.2169\/internalmedicine.7990-21","article-title":"Severe Asthma Where Eosinophilic Granulomatosis with Polyangiitis Became Apparent after the Discontinuation of Dupilumab","volume":"61","author":"Ikeda","year":"2022","journal-title":"Intern. Med."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1159\/000500544","article-title":"Anti-Neutrophil Cytoplasmic Antibodies Positivity and Anti-Leukotrienes in Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Monocentric Study on 134 Italian Patients","volume":"180","author":"Schroeder","year":"2019","journal-title":"Int. Arch. Allergy Immunol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1288","DOI":"10.1111\/j.1398-9995.2007.01486.x","article-title":"The common allergens in the Churg\u2013Strauss syndrome","volume":"62","author":"Bottero","year":"2007","journal-title":"Allergy"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"2553","DOI":"10.1007\/s10067-019-04538-w","article-title":"Clinical characteristics of Polish patients with ANCA-associated vasculitides\u2014Retrospective analysis of POLVAS registry","volume":"38","author":"Sznajd","year":"2019","journal-title":"Clin. Rheumatol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1055\/s-0038-1669454","article-title":"Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)","volume":"39","author":"Nguyen","year":"2018","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1615","DOI":"10.2147\/JAA.S542255","article-title":"Clinical, Biomarker, and Radiological Progression from Asthma to Systemic Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Cohort Study","volume":"18","author":"Lu","year":"2025","journal-title":"J. Asthma Allergy"},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Liu, S., Han, L., Liu, Y., Yang, J., Zhang, Y., Li, M., Tian, X., Zeng, X., Wang, L., and Zhang, F. (2022). Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis with Polyangiitis: Experience from a Longitudinal Chinese Cohort. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.885198"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1086","DOI":"10.1016\/j.chest.2019.11.045","article-title":"Eosinophilic Granulomatosis with Polyangiitis: Clinical Predictors of Long-term Asthma Severity","volume":"157","author":"Berti","year":"2020","journal-title":"Chest"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1080\/1744666X.2019.1697678","article-title":"Eosinophilic granulomatosis with polyangiitis: The multifaceted spectrum of clinical manifestations at different stages of the disease","volume":"16","author":"Berti","year":"2020","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.rmed.2018.07.017","article-title":"Severe\/uncontrolled asthma and overall survival in atopic patients with eosinophilic granulomatosis with polyangiitis","volume":"142","author":"Berti","year":"2018","journal-title":"Respir. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1007\/s10067-010-1530-3","article-title":"Pulmonary findings in Churg\u2013Strauss syndrome in chest X-rays and high resolution computed tomography at the time of initial diagnosis","volume":"29","author":"Szczeklik","year":"2010","journal-title":"Clin. Rheumatol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1094","DOI":"10.1002\/art.1780330806","article-title":"The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis)","volume":"33","author":"Masi","year":"1990","journal-title":"Arthritis Rheum."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1007\/s11886-025-02258-z","article-title":"Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis","volume":"27","author":"Srikantharajah","year":"2025","journal-title":"Curr. Cardiol. Rep."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"4538","DOI":"10.1093\/rheumatology\/keab027","article-title":"Heart disease in eosinophilic granulomatosis with polyangiitis (EGPA) patients: A screening approach proposal","volume":"60","author":"Harty","year":"2021","journal-title":"Rheumatology"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.ejim.2020.12.008","article-title":"Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): Prospective evaluation at a tertiary referral centre","volume":"85","author":"Zampieri","year":"2021","journal-title":"Eur. J. Intern. Med."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13075-024-03467-7","article-title":"Gastrointestinal lesions of eosinophilic granulomatosis with polyangiitis: A prediction model and clinical patterns","volume":"27","author":"Liu","year":"2025","journal-title":"Arthritis Res. Ther."},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Li, R., Chen, Y., Zhang, S., Peng, L., Zhou, J., Fei, Y., Zhang, W., Zhao, Y., and Zeng, X. (2022). Clinical characteristics and long-term outcome of patients with gastrointestinal involvement in eosinophilic granulomatosis with polyangiitis. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.1099722"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1007\/s00415-025-13203-z","article-title":"Central nervous system involvement and mimickers in ANCA associated vasculitis","volume":"272","author":"Esatoglu","year":"2025","journal-title":"J. Neurol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s40120-021-00315-7","article-title":"ANCA-Associated Vasculitic Neuropathies: A Review","volume":"11","author":"Koike","year":"2022","journal-title":"Neurol. Ther."},{"key":"ref_70","unstructured":"(2025). Multidisciplinary expert consensus on diagnosis and treatment of eosinophilic granulomatosis with polyangiitis (2025 Edition)]. Zhonghua Jie He He Hu Xi Za Zhi, 48, 418\u2013439."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1093\/rheumatology\/keaa416","article-title":"Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): A multicentric retrospective study of 63 biopsy-proven cases","volume":"60","author":"Durel","year":"2020","journal-title":"Rheumatology"},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Reggiani, F., L\u2019iMperio, V., Calatroni, M., Pagni, F., and Sinico, R.A. (2023). Renal involvement in eosinophilic granulomatosis with polyangiitis. Front. Med., 10.","DOI":"10.3389\/fmed.2023.1244651"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.ejim.2024.06.008","article-title":"Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA)","volume":"128","author":"Aparicio","year":"2024","journal-title":"Eur. J. Intern. Med."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1038\/s41584-025-01306-w","article-title":"Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis","volume":"21","author":"Merkel","year":"2025","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"59","DOI":"10.2500\/aap.2023.44.220072","article-title":"Eosinophilic granulomatosis with polyangiitis in allergic asthma: Efforts to make early diagnosis possible","volume":"44","author":"Zhao","year":"2023","journal-title":"Allergy Asthma Proc."},{"key":"ref_76","first-page":"710","article-title":"Insights from comparison of serum IgG4 between T2-eosinophilic asthma and eosinophilic granulomatosis with polyangiitis\/idiopathic hypereosinophilic syndrome","volume":"43","author":"Piga","year":"2025","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.3899\/jrheum.190093","article-title":"Evaluation of Potential Serum Biomarkers of Disease Activity in Diverse Forms of Vasculitis","volume":"47","author":"Warner","year":"2020","journal-title":"J. Rheumatol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"83","DOI":"10.55563\/clinexprheumatol\/08k9af","article-title":"Autoantibody profile in eosinophilic granulomatosis and polyangiitis: Predominance of anti-alpha-enolase antibodies","volume":"39","author":"Laskari","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1164\/rccm.201804-0809OC","article-title":"Sputum Antineutrophil Cytoplasmic Antibodies in Serum Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis with Polyangiitis","volume":"199","author":"Mukherjee","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_80","doi-asserted-by":"crossref","unstructured":"AlBloushi, S., and Al-Ahmad, M. (2024). Exploring the immunopathology of type 2 inflammatory airway diseases. Front. Immunol., 15.","DOI":"10.3389\/fimmu.2024.1285598"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"3087","DOI":"10.1111\/all.14620","article-title":"Adult-onset eosinophilic airway diseases","volume":"75","author":"Asano","year":"2020","journal-title":"Allergy"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"494","DOI":"10.2500\/aap.2022.43.220056","article-title":"Benralizumab in the management of rare primary eosinophilic lung diseases","volume":"43","author":"Soler","year":"2022","journal-title":"Allergy Asthma Proc."},{"key":"ref_83","doi-asserted-by":"crossref","unstructured":"Wang, C., He, R., Bai, X., Zhang, Y., Li, J., Zhao, J., Gao, W., and Guo, Q. (2025). Immune cell crosstalk between ANCA-associated vasculitis and IgG4-related disease: An unresolved pathogenic link. Front. Immunol., 16.","DOI":"10.3389\/fimmu.2025.1660956"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1415","DOI":"10.1016\/j.jaci.2023.03.011","article-title":"Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis","volume":"151","author":"Wechsler","year":"2023","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Choi, H.J., Ha, J.W., Song, J.J., Park, Y.-B., and Lee, S.-W. (2025). Overlap Syndrome of Primary Sj\u00f6gren Syndrome with Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis Based on the American College of Rheumatology (ACR)\/European Alliance of Associations for Rheumatology (EULAR) Criteria. Diagnostics, 15.","DOI":"10.3390\/diagnostics15091099"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1136\/ard-2022-223764","article-title":"EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update","volume":"83","author":"Hellmich","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1016\/j.ard.2025.06.2131","article-title":"Two-year efficacy and safety of anti-interleukin-5\/receptor therapy for eosinophilic granulomatosis with polyangiitis","volume":"84","author":"Merkel","year":"2025","journal-title":"Ann. Rheum. Dis."},{"key":"ref_88","doi-asserted-by":"crossref","unstructured":"Wilson-Morkeh, H., Seluk, L., Bosch, P., Aguiar, C., Thiel, J., Hellmich, B., Wechsler, M.E., and Siddiqui, S. (2026). Targeting Immunologic Pathways in Eosinophilic Granulomatosis with Polyangiitis: Translating Emerging Evidence into Clinical Practice. Allergy, Online ahead of print.","DOI":"10.1111\/all.70215"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1097\/BOR.0000000000001148","article-title":"Eosinophilic granulomatosis with polyangiitis: Recent therapeutic advances","volume":"38","author":"Chiara","year":"2026","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1093\/mr\/roae100","article-title":"Real-world safety and effectiveness of mepolizumab for patients with eosinophilic granulomatosis with polyangiitis in Japan: Long-term observation of the MARS study","volume":"35","author":"Ishii","year":"2025","journal-title":"Mod. Rheumatol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/art.41943","article-title":"Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis: A European Multicenter Observational Study","volume":"74","author":"Bettiol","year":"2022","journal-title":"Arthritis Rheumatol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"5108","DOI":"10.1093\/rheumatology\/keaf201","article-title":"Step-down treatment with mepolizumab for eosinophilic granulomatosis with polyangiitis: A real-life single-centre study","volume":"64","author":"Moroni","year":"2025","journal-title":"Rheumatology"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.anai.2024.11.004","article-title":"Real-world mepolizumab treatment in eosinophilic granulomatosis with polyangiitis reduces disease burden in the United States","volume":"134","author":"Mathur","year":"2025","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.ejim.2021.08.021","article-title":"Response to mepolizumab according to disease manifestations in patients with eosinophilic granulomatosis with polyangiitis","volume":"95","author":"Arismendi","year":"2022","journal-title":"Eur. J. Intern. Med."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/j.alit.2020.08.005","article-title":"The effects of mepolizumab on peripheral circulation and neurological symptoms in eosinophilic granulomatosis with polyangiitis (EGPA) patients","volume":"70","author":"Kitamura","year":"2021","journal-title":"Allergol. Int."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"01134","DOI":"10.1183\/23120541.01134-2025","article-title":"Benralizumab reduces sputum ANCA in patients with eosinophilic granulomatosis with polyangiitis","volume":"12","author":"Mukherjee","year":"2026","journal-title":"ERJ Open Res."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"103398","DOI":"10.1016\/j.jaut.2025.103398","article-title":"Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study","volume":"153","author":"Mattioli","year":"2025","journal-title":"J. Autoimmun."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"38","DOI":"10.2500\/aap.2025.46.240091","article-title":"Idiopathic non-mast cell angioedema: Treatment insights from global experts","volume":"46","author":"Christiansen","year":"2025","journal-title":"Allergy Asthma Proc."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1016\/j.anai.2021.01.035","article-title":"Efficacy and safety of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis","volume":"126","author":"Manka","year":"2021","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"1587","DOI":"10.1136\/ard-2023-224756","article-title":"Dupilumab for relapsing or refractory sinonasal and\/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: A European retrospective study","volume":"82","author":"Molina","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"165","DOI":"10.18176\/jiaci.0823","article-title":"Eosinophilia Induced by Blocking the IL-4\/IL-13 Pathway: Potential Mechanisms and Clinical Outcomes","volume":"32","author":"Olaguibel","year":"2022","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"2892","DOI":"10.1093\/rheumatology\/keae554","article-title":"Dupilumab for refractory chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis","volume":"64","author":"Padoan","year":"2024","journal-title":"Rheumatology"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/j.chest.2025.05.015","article-title":"Tezepelumab in Patients with Eosinophilic Granulomatosis with Polyangiitis after Suboptimal Response to Anti-IL-5\/5R Therapy","volume":"168","author":"Nanzer","year":"2025","journal-title":"Chest"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"103503","DOI":"10.1016\/j.autrev.2023.103503","article-title":"Combination of monoclonal antibodies targeting type 2 inflammation for severe asthma and eosinophilic granulomatosis with polyangiitis","volume":"23","author":"Davanzo","year":"2024","journal-title":"Autoimmun. Rev."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.1002\/art.41773","article-title":"2021 American College of Rheumatology\/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis","volume":"73","author":"Chung","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1056\/NEJMoa2311155","article-title":"Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis","volume":"390","author":"Wechsler","year":"2024","journal-title":"N. Engl. J. Med."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1002\/acr2.11263","article-title":"Clinical Manifestations and Long-Term Outcomes of Eosinophilic Granulomatosis with Polyangiitis in North America","volume":"3","author":"Doubelt","year":"2021","journal-title":"ACR Open Rheumatol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.jaip.2025.10.051","article-title":"Early Diagnosis and Treatment in Patients with Eosinophilic Granulomatosis with Polyangiitis","volume":"14","author":"Siddiqui","year":"2025","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1002\/art.37721","article-title":"Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort","volume":"65","author":"Comarmond","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1016\/j.autrev.2014.08.002","article-title":"Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors","volume":"13","author":"Samson","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1093\/rheumatology\/keu427","article-title":"Value of commonly measured laboratory tests as biomarkers of disease activity and predictors of relapse in eosinophilic granulomatosis with polyangiitis","volume":"54","author":"Grayson","year":"2014","journal-title":"Rheumatology"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1136\/ard.2011.155382","article-title":"IgG4 immune response in Churg\u2013Strauss syndrome","volume":"71","author":"Vaglio","year":"2012","journal-title":"Ann. Rheum. Dis."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1164\/rccm.202005-1628SO","article-title":"International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis","volume":"202","author":"Moiseev","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1711","DOI":"10.1002\/art.39614","article-title":"Trends in Long-Term Outcomes Among Patients with Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis with Renal Disease","volume":"68","author":"Rhee","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1097\/MD.0b013e318205a4c6","article-title":"The Five-Factor Score revisited: Assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort","volume":"90","author":"Guillevin","year":"2011","journal-title":"Medicine"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1093\/rheumatology\/kev286","article-title":"Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort","volume":"55","author":"Schirmer","year":"2015","journal-title":"Rheumatology"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1136\/annrheumdis-2012-201531","article-title":"A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg\u2013Strauss, EGPA): Monocentric experiences in 150 patients","volume":"72","author":"Moosig","year":"2013","journal-title":"Ann. Rheum. Dis."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1016\/j.semarthrit.2021.07.001","article-title":"Developing a composite outcome tool to measure response to treatment in ANCA-associated vasculitis: A mixed methods study from OMERACT 2020","volume":"51","author":"Quinn","year":"2021","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1016\/j.semarthrit.2020.09.010","article-title":"Use and reporting of outcome measures in randomized trials for anti-neutrophil cytoplasmic antibody-associated vasculitis: A systematic literature review","volume":"50","author":"Monti","year":"2020","journal-title":"Semin. Arthritis Rheum."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/16\/11\/1734\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T12:13:10Z","timestamp":1780575190000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/16\/11\/1734"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,4]]},"references-count":119,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2026,6]]}},"alternative-id":["diagnostics16111734"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics16111734","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,4]]}}}