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Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65: 1-11, 2013.","DOI":"10.1007\/s10157-013-0869-6"},{"key":"2","doi-asserted-by":"publisher","unstructured":"2. Asano K, Ueki S, Tamari M, Imoto Y, Fujieda S, Taniguchi M. Adult-onset eosinophilic airway diseases. Allergy 75: 3087-3099, 2020.","DOI":"10.1111\/all.14620"},{"key":"3","unstructured":"3. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 27: 277-301, 1951."},{"key":"4","doi-asserted-by":"crossref","unstructured":"4. Weller PF, Spencer LA. Functions of tissue-resident eosinophils. Nat Rev Immunol 17: 746-760, 2017.","DOI":"10.1038\/nri.2017.95"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5. Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 90: 19-27, 2011.","DOI":"10.1097\/MD.0b013e318205a4c6"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 65: 270-281, 2013.","DOI":"10.1002\/art.37721"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. Li R, Chen Y, Zhang S, et al. Clinical characteristics and long-term outcome of patients with gastrointestinal involvement in eosinophilic granulomatosis with polyangiitis. Front Immunol 13: 1099722, 2022.","DOI":"10.3389\/fimmu.2022.1099722"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8. Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 52: 2926-2935, 2005.","DOI":"10.1002\/art.21250"},{"key":"9","doi-asserted-by":"publisher","unstructured":"9. Furuta S, Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis. Allergology International 68: 430-436, 2019.","DOI":"10.1016\/j.alit.2019.06.004"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10. Rothenberg ME, Hogan SP. The eosinophil. Annu Rev Immunol 24: 147-174, 2006.","DOI":"10.1146\/annurev.immunol.24.021605.090720"},{"key":"11","doi-asserted-by":"crossref","unstructured":"11. Nagase H, Ueki S, Fujieda S. The roles of IL-5 and anti-IL-5 treatment in eosinophilic diseases: Asthma, eosinophilic granulomatosis with polyangiitis, and eosinophilic chronic rhinosinusitis. Allergol Int 69: 178-186, 2020.","DOI":"10.1016\/j.alit.2020.02.002"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12. Gleich GJ. Mechanisms of eosinophil-associated inflammation. J Allergy Clin Immunol 105: 651-663, 2000.","DOI":"10.1067\/mai.2000.105712"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Khoury P, Grayson PC, Klion AD. Eosinophils in vasculitis: characteristics and roles in pathogenesis. Nat Rev Rheumatol 10: 474-483, 2014.","DOI":"10.1038\/nrrheum.2014.98"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. Fijolek J, Radzikowska E. Eosinophilic granulomatosis with polyangiitis - Advances in pathogenesis, diagnosis, and treatment. Front Med (Lausanne) 10: 1145257, 2023.","DOI":"10.3389\/fmed.2023.1145257"},{"key":"15","doi-asserted-by":"crossref","unstructured":"15. Bond M, Fagni F, Moretti M, et al. At the Heart of Eosinophilic Granulomatosis with Polyangiitis: into Cardiac and Vascular Involvement. Curr Rheumatol Rep 24: 337-351, 2022.","DOI":"10.1007\/s11926-022-01087-1"},{"key":"16","doi-asserted-by":"publisher","unstructured":"16. Ueki S, Melo RCN, Ghiran I, Spencer LA, Dvorak AM, Weller PF. Eosinophil extracellular DNA trap cell death mediates lytic release of free secretion-competent eosinophil granules in humans. Blood 121: 2074-2083, 2013.","DOI":"10.1182\/blood-2012-05-432088"},{"key":"17","doi-asserted-by":"crossref","unstructured":"17. Ueki S, Konno Y, Takeda M, et al. Eosinophil extracellular trap cell death-derived DNA traps: Their presence in secretions and functional attributes. J Allergy Clin Immunol 137: 258-267, 2016.","DOI":"10.1016\/j.jaci.2015.04.041"},{"key":"18","doi-asserted-by":"crossref","unstructured":"18. Fukuchi M, Miyabe Y, Furutani C, et al. How to detect eosinophil ETosis (EETosis) and extracellular traps. Allergol Int 70: 19-29, 2021.","DOI":"10.1016\/j.alit.2020.10.002"},{"key":"19","doi-asserted-by":"crossref","unstructured":"19. Fukuchi M, Kamide Y, Ueki S, et al. Eosinophil ETosis-Mediated Release of Galectin-10 in Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol 73: 1683-1693, 2021.","DOI":"10.1002\/art.41727"},{"key":"20","doi-asserted-by":"crossref","unstructured":"20. Kawakami T, Yokoyama K, Ikeda T, Tomizawa H, Ueki S. Presence of eosinophil extracellular trap cell death in the affected skin of eosinophilic granulomatosis with polyangiitis. J Dermatol 50: 551-555, 2023.","DOI":"10.1111\/1346-8138.16656"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21. Koike H, Nishi R, Furukawa S, et al. In vivo visualization of eosinophil secretion in eosinophilic granulomatosis with polyangiitis: An ultrastructural study. Allergol Int 71: 373-382, 2022.","DOI":"10.1016\/j.alit.2022.02.009"},{"key":"22","doi-asserted-by":"crossref","unstructured":"22. Hasegawa E, Iijima M, Sekine A, et al. A case of eosinophilic granulomatosis with polyangiitis preceded by allergic bullous lesions. Mod Rheumatol Case Rep 9: 143-148, 2025.","DOI":"10.1093\/mrcr\/rxae046"},{"key":"23","doi-asserted-by":"crossref","unstructured":"23. Hayashi S, Sugiura T, Hakamata S, et al. Charcot-Leyden crystals in kidney. Kidney Int 104: 206, 2023.","DOI":"10.1016\/j.kint.2023.02.007"},{"key":"24","doi-asserted-by":"crossref","unstructured":"24. Ueki S, Miyabe Y, Yamamoto Y, et al. Charcot-Leyden Crystals in Eosinophilic Inflammation: Active Cytolysis Leads to Crystal Formation. Curr Allergy Asthma Rep 19: 35, 2019.","DOI":"10.1007\/s11882-019-0868-0"},{"key":"25","doi-asserted-by":"publisher","unstructured":"25. Tomizawa H, Yamada Y, Arima M, et al. Galectin-10 as a Potential Biomarker for Eosinophilic Diseases. Biomolecules 12: 1385, 2022.","DOI":"10.3390\/biom12101385"},{"key":"26","unstructured":"26. Bettiol A, Sinico RA, Schiavon F, et al. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Eur Respir J 57: 2004158, 2021."},{"key":"27","doi-asserted-by":"crossref","unstructured":"27. Hashimoto T, Ueki S, Kamide Y, et al. Increased Circulating Cell-Free DNA in Eosinophilic Granulomatosis With Polyangiitis: Implications for Eosinophil Extracellular Traps and Immunothrombosis. Front Immunol 12: 801897, 2021.","DOI":"10.3389\/fimmu.2021.801897"},{"key":"28","doi-asserted-by":"crossref","unstructured":"28. Vaglio A, Casazza I, Grasselli C, Corradi D, Sinico RA, Buzio C. Churg-Strauss syndrome. Kidney Int 76: 1006-1011, 2009.","DOI":"10.1038\/ki.2009.210"},{"key":"29","doi-asserted-by":"crossref","unstructured":"29. Takeuchi H, Kawamura K, Kawasaki T, Oka N. Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis. Front Neurol 13: 1057767, 2022.","DOI":"10.3389\/fneur.2022.1057767"},{"key":"30","doi-asserted-by":"crossref","unstructured":"30. Dejaco C, Oppl B, Monach P, et al. Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss). PLoS One 10: e0121737, 2015.","DOI":"10.1371\/journal.pone.0121737"},{"key":"31","doi-asserted-by":"crossref","unstructured":"31. Jakiela B, Szczeklik W, Plutecka H, et al. Increased production of IL-5 and dominant Th2-type response in airways of Churg-Strauss syndrome patients. Rheumatology (Oxford) 51: 1887-1893, 2012.","DOI":"10.1093\/rheumatology\/kes171"},{"key":"32","doi-asserted-by":"crossref","unstructured":"32. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med 376: 1921-1932, 2017.","DOI":"10.1056\/NEJMoa1702079"},{"key":"33","doi-asserted-by":"crossref","unstructured":"33. Wechsler ME, Nair P, Terrier B, et al. Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med 390: 911-921, 2024.","DOI":"10.1056\/NEJMoa2311155"},{"key":"34","doi-asserted-by":"crossref","first-page":"e143366","DOI":"10.1172\/jci.insight.143366","volume":"6","author":"ME Kotas","year":"2021","unstructured":"34. Kotas ME, Dion J, Van Dyken S, et al. A role for IL-33-activated ILC2s in eosinophilic vasculitis. JCI Insight 6: e143366, 143366, 2021.","journal-title":"JCI Insight","ISSN":"https:\/\/id.crossref.org\/issn\/2379-3708","issn-type":"print"},{"key":"35","unstructured":"35. Jakiela B, Sanak M, Szczeklik W, et al. Both Th2 and Th17 responses are involved in the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol 29 (1 Suppl 64): S23-S34, 2011."},{"key":"36","doi-asserted-by":"crossref","unstructured":"36. Fujioka A, Yamamoto T, Takasu H, et al. The analysis of mRNA expression of cytokines from skin lesions in Churg-Strauss syndrome. J Dermatol 25: 171-177, 1998.","DOI":"10.1111\/j.1346-8138.1998.tb02375.x"},{"key":"37","unstructured":"37. Lyons PA, Peters JE, Alberici F, et al. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status. Nat Commun 10: 5120, 2019."},{"key":"38","unstructured":"38. Rout P, Maher L. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome). In: <i>StatPearls<\/i>. StatPearls Publishing; 2025 [Internet]. [cited 2026 Mar 12]. Available from: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK537099\/"},{"key":"39","doi-asserted-by":"crossref","unstructured":"39. Rubenstein E, Maldini C, Vaglio A, et al. Cluster Analysis to Explore Clinical Subphenotypes of Eosinophilic Granulomatosis With Polyangiitis. J Rheumatol 50: 1446-1453, 2023.","DOI":"10.3899\/jrheum.2023-0325"},{"key":"40","unstructured":"40. Natorska J, Z\u0105bczyk M, Siudut J, Krawiec P, Mastalerz L, Undas A. Neutrophil extracellular traps formation in patients with eosinophilic granulomatosis with polyangiitis: association with eosinophilic inflammation. Clin Exp Rheumatol 35 Suppl 103: 27-32, 2017."},{"key":"41","doi-asserted-by":"crossref","unstructured":"41. Shiratori-Aso S, Nakazawa D. The involvement of NETs in ANCA-associated vasculitis. Front Immunol 14: 1261151, 2023.","DOI":"10.3389\/fimmu.2023.1261151"},{"key":"42","doi-asserted-by":"crossref","unstructured":"42. Mukherjee M, Thomas SR, Radford K, et al. Sputum Antineutrophil Cytoplasmic Antibodies in Serum Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis with Polyangiitis. Am J Respir Crit Care Med 199: 158-170, 2019.","DOI":"10.1164\/rccm.201804-0809OC"},{"key":"43","doi-asserted-by":"crossref","unstructured":"43. Dong C, Lu B, Zhong C, et al. Airway immune profiles and therapeutic implications of IGF1 in eosinophilic granulomatosis with polyangiitis. Nat Commun 17: 1346, 2026.","DOI":"10.1038\/s41467-025-68104-6"},{"key":"44","doi-asserted-by":"crossref","unstructured":"44. Fuchs TA, Brill A, Duerschmied D, et al. Extracellular DNA traps promote thrombosis. Proc Natl Acad Sci U S A 107: 15880-15885, 2010.","DOI":"10.1073\/pnas.1005743107"},{"key":"45","doi-asserted-by":"crossref","unstructured":"45. Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol 19: 25-32, 2007.","DOI":"10.1097\/BOR.0b013e3280119854"},{"key":"46","doi-asserted-by":"crossref","unstructured":"46. Healy B, Bibby S, Steele R, Weatherall M, Nelson H, Beasley R. Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome. J Allergy Clin Immunol 131: 571-576.e1-e6, 2013.","DOI":"10.1016\/j.jaci.2012.05.058"},{"key":"47","doi-asserted-by":"crossref","unstructured":"47. Ueki S, Yamauchi H, Takeda M, Chihara J. Eosinophilic granuloma of the middle ear. J Rheumatol 38: 2005-2006, 2011.","DOI":"10.3899\/jrheum.110126"},{"key":"48","doi-asserted-by":"crossref","unstructured":"48. Emmi G, Bettiol A, Gelain E, et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 19: 378-393, 2023.","DOI":"10.1038\/s41584-023-00958-w"},{"key":"49","doi-asserted-by":"crossref","unstructured":"49. Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 26: 545-553, 2015.","DOI":"10.1016\/j.ejim.2015.04.022"},{"key":"50","doi-asserted-by":"crossref","unstructured":"50. Matucci A, Vivarelli E, Perlato M, et al. EGPA Phenotyping: Not Only ANCA, but Also Eosinophils. Biomedicines 11: 776, 2023.","DOI":"10.3390\/biomedicines11030776"},{"key":"51","doi-asserted-by":"crossref","unstructured":"51. Guilpain P, Auclair JF, Tamby MC, et al. Serum eosinophil cationic protein: a marker of disease activity in Churg-Strauss syndrome. Ann N Y Acad Sci 1107: 392-399, 2007.","DOI":"10.1196\/annals.1381.041"},{"key":"52","doi-asserted-by":"crossref","unstructured":"52. Latorre M, Seccia V, Puxeddu I, et al. Severe Eosinophilic Asthma or Eosinophilic Granulomatosis With Polyangiitis: Potential Biomarkers for Novel Diagnostic Strategies. J Allergy Clin Immunol Pract 12: 3057-3067, 2024.","DOI":"10.1016\/j.jaip.2024.08.011"},{"key":"53","doi-asserted-by":"crossref","unstructured":"53. Dallos T, Heiland GR, Strehl J, et al. CCL17\/thymus and activation-related chemokine in Churg-Strauss syndrome. Arthritis Rheum 62: 3496-3503, 2010.","DOI":"10.1002\/art.27678"},{"key":"54","doi-asserted-by":"crossref","unstructured":"54. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis 81: 309-314, 2022.","DOI":"10.1002\/art.41982"},{"key":"55","doi-asserted-by":"crossref","unstructured":"55. Trivioli G, Marquez A, Martorana D, et al. Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management. Nat Rev Rheumatol 18: 559-574, 2022.","DOI":"10.1038\/s41584-022-00819-y"},{"key":"56","doi-asserted-by":"crossref","unstructured":"56. Tamaki H. Current definition of remission in eosinophilic granulomatosis with polyangiitis (EGPA) and future perspectives. Allergol Int 75: 26-31, 2026.","DOI":"10.1016\/j.alit.2025.10.004"},{"key":"57","doi-asserted-by":"crossref","unstructured":"57. Mukhtyar C, Lee R, Brown D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 68: 1827-1832, 2009.","DOI":"10.1136\/ard.2008.101279"},{"key":"58","unstructured":"58. \u597d\u9178\u7403\u6027\u591a\u767a\u8840\u7ba1\u708e\u6027\u8089\u82bd\u816b\u75c7.pdf [Internet]. [cited 2025 Dec 31]. Available from: https:\/\/www.mhlw.go.jp\/content\/10905000\/001173549.pdf"},{"key":"59","doi-asserted-by":"crossref","unstructured":"59. Jia WY, Zhang JJ. Effects of glucocorticoids on leukocytes: Genomic and non-genomic mechanisms. World J Clin Cases 10: 7187-7194, 2022.","DOI":"10.12998\/wjcc.v10.i21.7187"},{"key":"60","unstructured":"60. Biddle K, Jade J, Wilson-Morkeh H, et al. The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis. Rheumatology (Oxford) 64: 4470-4494, 2025."},{"key":"61","doi-asserted-by":"crossref","unstructured":"61. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology\/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol 73: 1366-1383, 2021.","DOI":"10.1002\/art.41773"},{"key":"62","doi-asserted-by":"crossref","unstructured":"62. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis 83: 30-47, 2024.","DOI":"10.1136\/ard-2024-225606"},{"key":"63","doi-asserted-by":"crossref","unstructured":"63. Casal Moura M, Merkel PA, Jayne D, et al. Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis. Nat Rev Rheumatol 21: 719-736, 2025.","DOI":"10.1038\/s41584-025-01306-w"},{"key":"64","doi-asserted-by":"crossref","unstructured":"64. Gopaluni S, Jayne D. Clinical Trials in Vasculitis. Curr Treatm Opt Rheumatol 2: 161-177, 2016.","DOI":"10.1007\/s40674-016-0045-8"},{"key":"65","doi-asserted-by":"crossref","unstructured":"65. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 75: 17-28, 1996.","DOI":"10.1097\/00005792-199601000-00003"},{"key":"66","doi-asserted-by":"crossref","unstructured":"66. Cohen P, Pagnoux C, Mahr A, et al. Churg-Strauss syndrome with poor-prognosis factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum 57: 686-693, 2007.","DOI":"10.1002\/art.22679"},{"key":"67","doi-asserted-by":"crossref","unstructured":"67. Sartorelli S, Chassagnon G, Cohen P, et al. Revisiting characteristics, treatment and outcome of cardiomyopathy in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Rheumatology (Oxford) 61: 1175-1184, 2022.","DOI":"10.1093\/rheumatology\/keab514"},{"key":"68","doi-asserted-by":"crossref","unstructured":"68. Sorin B, Papo M, Sinico RA, et al. Glucocorticoids versus glucocorticoids plus cyclophosphamide in eosinophilic granulomatosis with polyangiitis with poor-prognosis factors. J Autoimmun 149: 103338, 2024.","DOI":"10.1016\/j.jaut.2024.103338"},{"key":"69","doi-asserted-by":"crossref","unstructured":"69. Sorin B, Papo M, Sinico RA, et al. Glucocorticoids vs glucocorticoids plus cyclophosphamide in eosinophilic granulomatosis with polyangiitis without poor-prognosis factors: a target trial emulation study. Rheumatology (Oxford) 64: 5238-5244, 2025.","DOI":"10.1093\/rheumatology\/keaf256"},{"key":"70","doi-asserted-by":"crossref","unstructured":"70. Terrier B, Pugnet G, de Moreuil C, et al. Rituximab Versus Conventional Therapy for Remission Induction in Eosinophilic Granulomatosis With Polyangiitis: A Randomized Controlled Trial. Ann Intern Med 178: 1249-1257, 2025.","DOI":"10.7326\/ANNALS-24-03947"},{"key":"71","doi-asserted-by":"publisher","unstructured":"71. Teixeira V, Mohammad AJ, Jones RB, Smith R, Jayne D. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open 5: e000905, 2019.","DOI":"10.1136\/rmdopen-2019-000905"},{"key":"72","doi-asserted-by":"crossref","unstructured":"72. Haldar P, Brightling CE, Hargadon B, et al. Mepolizumab and exacerbations of refractory eosinophilic asthma. N Engl J Med 360: 973-984, 2009.","DOI":"10.1056\/NEJMoa0808991"},{"key":"73","doi-asserted-by":"crossref","unstructured":"73. Nair P, Pizzichini MMM, Kjarsgaard M, et al. Mepolizumab for prednisone-dependent asthma with sputum eosinophilia. N Engl J Med 360: 985-993, 2009.","DOI":"10.1056\/NEJMoa0805435"},{"key":"74","doi-asserted-by":"crossref","unstructured":"74. Ueki S. Through the MIRRA and what we found there. ERJ Open Res 10: 00825-02023, 2024.","DOI":"10.1183\/23120541.00825-2023"},{"key":"75","doi-asserted-by":"crossref","unstructured":"75. Ishii T, Kunishige H, Kobayashi T, et al. Real-world safety and effectiveness of mepolizumab for patients with eosinophilic granulomatosis with polyangiitis in Japan: A 48-week interim analysis of the MARS study. Mod Rheumatol 34: 978-987, 2024.","DOI":"10.1093\/mr\/road109"},{"key":"76","doi-asserted-by":"crossref","unstructured":"76. Bettiol A, Urban ML, Dagna L, et al. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study. Arthritis Rheumatol 74: 295-306, 2022.","DOI":"10.1002\/art.41943"},{"key":"77","unstructured":"77. Assistance Publique - H\u00f4pitaux de Paris. <i>Evaluation of MEpolizumab-Based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-Blind Study<\/i>. clinicaltrials.gov; 2025 [Internet]. [cited 2026 Jan 3]. Available from: https:\/\/clinicaltrials.gov\/study\/NCT05030155"},{"key":"78","doi-asserted-by":"crossref","unstructured":"78. Moran AM, Ramakrishnan S, Borg CA, et al. Blood Eosinophil Depletion with Mepolizumab, Benralizumab, and Prednisolone in Eosinophilic Asthma. Am J Respir Crit Care Med 202: 1314-1316, 2020.","DOI":"10.1164\/rccm.202003-0729LE"},{"key":"79","doi-asserted-by":"crossref","unstructured":"79. Wechsler ME, Agmon-Levin N, Jayne DRW, et al. Discontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab. ACR Open Rheumatol 7: e70096, 2025.","DOI":"10.1002\/acr2.70096"},{"key":"80","doi-asserted-by":"crossref","unstructured":"80. Spataro F, Solimando AG, Di Girolamo A, Vacca A, Ria R. Efficacy and safety of benralizumab in eosinophilic granulomatosis with polyangiitis: A meta-analysis of eight studies. Eur J Clin Invest 55: e14333, 2025.","DOI":"10.1111\/eci.14333"},{"key":"81","doi-asserted-by":"crossref","unstructured":"81. Merkel PA, Nair PK, Khalidi N, et al. Two-year efficacy and safety of anti-interleukin-5\/receptor therapy for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis 84: 1888-1899, 2025.","DOI":"10.1016\/j.ard.2025.06.2131"},{"key":"82","doi-asserted-by":"crossref","unstructured":"82. Zwiers E, Montizaan D, Kip A, et al. Inhibition of EETosis with an anti-citrullinated histone antibody: a novel therapeutic approach for eosinophilic inflammatory disorders. Front Immunol 16: 1533407, 2025.","DOI":"10.3389\/fimmu.2025.1533407"},{"key":"83","doi-asserted-by":"crossref","unstructured":"83. Drynda A, Padjas A, W\u00f3jcik K, et al. Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry. J Immunol Res 2024: 4283928, 2024.","DOI":"10.1155\/2024\/4283928"},{"key":"84","doi-asserted-by":"crossref","unstructured":"84. Kamide Y, Sonehara K, Sekiya K, et al. Bioactive Mediator Profile of Mepolizumab-Treated Eosinophilic Granulomatosis With Polyangiitis. Allergy 80: 882-885, 2025.","DOI":"10.1111\/all.16395"},{"key":"85","doi-asserted-by":"crossref","unstructured":"85. Nair PK, Hellmich B, Bourdin A, et al. The Effect of Benralizumab and Mepolizumab on Use of Oral Glucocorticoids in Patients With Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol Published online September 2025. Online ahead of print.","DOI":"10.1002\/art.43398"},{"key":"86","doi-asserted-by":"crossref","unstructured":"86. Wechsler ME, Silver J, Wolff G, et al. Long-Term Safety and Efficacy of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol 77: 1052-1062, 2025.","DOI":"10.1002\/art.43146"},{"key":"87","doi-asserted-by":"crossref","unstructured":"87. Le Floc&apos;h A, Allinne J, Nagashima K, et al. Dual blockade of IL-4 and IL-13 with dupilumab, an IL-4R\u03b1 antibody, is required to broadly inhibit type 2 inflammation. Allergy 75: 1188-1204, 2020.","DOI":"10.1111\/all.14151"},{"key":"88","doi-asserted-by":"crossref","unstructured":"88. Suzaki I, Ito K, Ueki S, et al. Dupilumab Decreases Local Galectin-10 Expression in Eosinophilic Chronic Rhinosinusitis. Int Forum Allergy Rhinol 15: 876-880, 2025.","DOI":"10.1002\/alr.23614"},{"key":"89","doi-asserted-by":"crossref","unstructured":"89. Galant-Swafford J, Geng B, Leibel S, et al. Two pediatric cases of ANCA-negative eosinophilic granulomatosis with polyangiitis successfully treated with dupilumab. J Allergy Clin Immunol Pract 8: 3643-3646.e1, 2020.","DOI":"10.1016\/j.jaip.2020.06.052"},{"key":"90","doi-asserted-by":"crossref","unstructured":"90. Yamazaki K, Nomizo T, Hatanaka K, Hayama N, Oguma T, Asano K. Eosinophilic granulomatosis with polyangiitis after treatment with dupilumab. J Allergy Clin Immunol Glob 1: 180-182, 2022.","DOI":"10.1016\/j.jacig.2022.03.006"},{"key":"91","doi-asserted-by":"crossref","unstructured":"91. Suzaki I, Tanaka A, Yanai R, et al. Eosinophilic granulomatosis with polyangiitis developed after dupilumab administration in patients with eosinophilic chronic rhinosinusitis and asthma: a case report. BMC Pulm Med 23: 130, 2023.","DOI":"10.1186\/s12890-023-02415-6"},{"key":"92","doi-asserted-by":"crossref","unstructured":"92. Jayne DRW, Merkel PA, Schall TJ, Bekker P; ADVOCATE Study Group. Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med 384: 599-609, 2021.","DOI":"10.1056\/NEJMoa2023386"},{"key":"93","doi-asserted-by":"crossref","unstructured":"93. Hellmark T, Ohlsson S, Pettersson \u00c5, Hansson M, Johansson \u00c5CM. Eosinophils in anti-neutrophil cytoplasmic antibody associated vasculitis. BMC Rheumatol 3: 9, 2019.","DOI":"10.1186\/s41927-019-0059-6"},{"key":"94","doi-asserted-by":"crossref","unstructured":"94. L\u00f3pez Paraja M, Starita Fajardo G, Donate Velasco I, et al. Molecular Pathogenesis and Targeted Therapies in Eosinophilic Granulomatosis with Polyangiitis: An Updated Review. 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