Table Info

Table 2.

Benralizumab therapy in patients with EGPA

Reference	Study design	Number of patients	Dose and route of administration	Results
Takenaka et al. [44], 2019	Case report	1	30 mg SC	Blood eosinophil normalization Clinical remission CS reduction MPO-ANCA negativation
Padoan et al. [56], 2020	Case series	5	Initial 3 doses: 30 mg SC every 4 weeks Maintenance dose: 30 mg SC every 8 weeks	3 Patients completely withdraw from CS Complete depletion in blood eosinophils ACT improvement No statistically significant difference in lung function Lack of improvement in ANCA-status
Nanzer et al. [57], 2020	Case series	11	Initial 3 doses: 30 mg SC every 4 weeks Maintenance dose: 30 mg SC every 8 weeks	CS reduction Complete depletion in blood eosinophils BVAS improvement No statistically significant difference in lung function ACT improvement Quality of life improvement
Guntur et al. [58], 2021	Prospective open-label pilot study	10	Initial 3 doses: 30 mg SC every 4 weeks Maintenance dose: 30 mg SC every 8 weeks	Benralizumab was well tolerated 50% Of the patients could completely withdraw from CS Significant decrease in absolute blood eosinophils No significant differences in lung function, BVAS, and quality of life

Declarations

Author contributions

ACH: Conceptualization, Investigation, Writing—original draft, Writing—review & editing. CP: Conceptualization, Investigation, Visualization, Writing—review & editing. GP: Validation, Writing—review & editing, Supervision. All authors read and approved the submitted version.

Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical approval

Not applicable.

Consent to participate

Not applicable.

Consent to publication

Not applicable.

Availability of data and materials

Not applicable.

Funding

Not applicable.

Copyright

References

Olivieri B, Tinazzi E, Caminati M, Lunardi C. Biologics for the treatment of allergic conditions: eosinophil disorders. Immunol Allergy Clin North Am. 2020;40:649–65. [DOI] [PubMed]

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised international Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11. [DOI] [PubMed]

Loughlin JE, Cole JA, Rothman KJ, Johnson ES. Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients. Ann Allergy Asthma Immunol. 2002;88:319–25. [DOI] [PubMed]

Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100. [DOI] [PubMed]

Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al.; DCVAS Study Group. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Arthritis Rheum. 2022;74:386–92. [DOI] [PubMed]

Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68:1827–32. [DOI] [PubMed]

Chaigne B, Terrier B, Thieblemont N, Witko-Sarsat V, Mouthon L. Dividing the Janus vasculitis? Pathophysiology of eosinophilic granulomatosis with polyangitis. Autoimmun Rev. 2016;15:139–45. [DOI] [PubMed]

Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, Lazor R, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): a study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017;16:1–9. [DOI] [PubMed]

Raffray L, Guillevin L. Updates for the treatment of EGPA. Presse Med. 2020;49:104036. [DOI] [PubMed]

10.

Rothenberg ME, Hogan SP. The eosinophil. Annu Rev Immunol. 2006;24:147–74. [DOI] [PubMed]

11.

Nappi E, De Santis M, Paoletti G, Pelaia C, Terenghi F, Pini D, et al. New onset of eosinophilic granulomatosis with polyangiitis following mRNA-based COVID-19 vaccine. Vaccines (Basel). 2022;10:716. [DOI] [PubMed] [PMC]

12.

Bayrak Durmaz MS, Çelebi Sözener Z, Bavbek S. Eosinophilic granulomatosis with polyangitis: a new target for biologicals. Tuberk Toraks. 2022;70:93–101. [DOI] [PubMed]

13.

Erle DJ, Sheppard D. The cell biology of asthma. J Cell Biol. 2014;205:621–31. [DOI] [PubMed] [PMC]

14.

Nguyen Y, Guillevin L. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Semin Respir Crit Care Med. 2018;39:471–81. [DOI] [PubMed]

15.

Hellmich B, Csernok E, Gross WL. Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome. Ann N Y Acad Sci. 2005;1051:121–31. [DOI] [PubMed]

16.

Marvisi C, Sinico RA, Salvarani C, Jayne D, Prisco D, Terrier B, et al. New perspectives in eosinophilic granulomatosis with polyangiitis (EGPA): report of the first meeting of the European EGPA Study Group. Intern Emerg Med. 2019;14:1193–7. [DOI] [PubMed]

17.

Jakiela B, Szczeklik W, Plutecka H, Sokolowska B, Mastalerz L, Sanak M, et al. Increased production of IL-5 and dominant Th2-type response in airways of Churg-Strauss syndrome patients. Rheumatology (Oxford). 2012;51:1887–93. [DOI] [PubMed]

18.

Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90:19–27. [DOI] [PubMed]

19.

Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. 1977;52:477–84. [PubMed]

20.

Guillevin L, Jarrousse B, Lok C, Lhote F, Jais JP, Le Thi Huong Du D, et al. Longterm followup after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa. J Rheumatol. 1991;18:567–74. [PubMed]

21.

Vega Villanueva KL, Espinoza LR. Eosinophilic Vasculitis. Curr Rheumatol Rep. 2020;22:5. [DOI] [PubMed]

22.

Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERAEDTA recommendations for the management of ANC-aassociated vasculitis. Ann Rheum Dis. 2016;75:1583–94. Erratum in: Ann Rheum Dis. 2017;76:1480. [DOI] [PubMed]

23.

Emejuaiwe N. Treatment strategies in ANCA-associated vasculitis. Curr Rheumatol Rep. 2019;21:33. [DOI] [PubMed]

24.

Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al.; EGPA Mepolizumab Study Team. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017;376:1921–32. [DOI] [PubMed] [PMC]

25.

Flood-Page P, Menzies-Gow A, Phipps S, Ying S, Wangoo A, Ludwig MS, et al. Anti-IL-5 treatment reduces deposition of ECM proteins in the bronchial subepithelial basement membrane of mild atopic asthmatics. J Clin Invest. 2003;112:1029–36. [DOI] [PubMed] [PMC]

26.

Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, et al.; MENSA Investigators. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014;371:1198–207. [DOI] [PubMed]

27.

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. 2020;69:178–86. [DOI] [PubMed]

28.

Highlights of prescribing information [Internet]. Silver Spring: U.S. Food and Drug Administration; c2022 [cited 2022 Sep 16]. Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/761122s000lbl.pdf

29.

Keating GM. Mepolizumab: first global approval. Drugs. 2015;75:2163–9. [DOI] [PubMed]

30.

Kahn JE, Grandpeix-Guyodo C, Marroun I, Catherinot E, Mellot F, Roufosse F, et al. Sustained response to mepolizumab in refractory Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:267–70. [DOI] [PubMed]

31.

Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:1336–43. [DOI] [PubMed]

32.

Herrmann K, Gross WL, Moosig F. Extended follow-up after stopping mepolizumab in relapsing/refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2012;30:S62–5. [PubMed]

33.

Steinfeld J, Bradford ES, Brown J, Mallett S, Yancey SW, Akuthota P, et al. Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol. 2019;143:2170–7. [DOI] [PubMed] [PMC]

34.

Caminati M, Crisafulli E, Lunardi C, Micheletto C, Festi G, Maule M, et al. Mepolizumab 100 mg in severe asthmatic patients with EGPA in remission phase. J Allergy Clin Immunol Pract. 2021;9:1386–8. [DOI] [PubMed]

35.

Özdel Öztürk B, Yavuz Z, Aydın Ö, Mungan D, Sin BA, Demirel YS. Effectiveness of low-dose mepolizumab in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA): a real-life experience. Int Arch Allergy Immunol. 2022;183:1281–90. [DOI] [PubMed]

36.

Bettiol A, Urban ML, Dagna L, Cottin V, Franceschini F, Del Giacco S, et al.; European EGPA Study Group. Mepolizumab for eosinophilic granulomatosis with polyangiitis: a European multicenter observational study. Arthritis Rheumatol. 2022;74:295–306. [DOI] [PubMed] [PMC]

37.

Study of mepolizumab-based regimen compared to conventional therapy strategy in patients with eosinophilic granulomatosis with polyangiitis (E-merge) (E-merge) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 29]. Available from: https://clinicaltrials.gov/ct2/show/NCT05030155?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=2&rank=5

38.

Mepolizumab long-term study to asses real world safety and effectiveness of eosinophilic granulomatosis with polyangiitis (EGPA) in Japan (MARS) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 29]. Available from: https://clinicaltrials.gov/ct2/show/NCT04551989?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=2&rank=7

39.

Long-term access program (LAP) of mepolizumab who participated in study MEA115921 [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 29]. Available from: https://clinicaltrials.gov/ct2/show/NCT03298061?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=3&rank=15

40.

NUCALA^® special drug use investigation (EGPA, long-term) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 29]. Available from: https://clinicaltrials.gov/ct2/show/NCT03557060?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=3&rank=16

41.

Bleecker ER, FitzGerald JM, Chanez P, Papi A, Weinstein SF, Barker P, et al.; SIROCCO study investigators. Efficacy and safety of benralizumab for patients with severe asthma uncontrolled with high-dosage inhaled corticosteroids and long-acting β₂-agonists (SIROCCO): a randomised, multicentre, placebo-controlled phase 3 trial. Lancet. 2016;388:2115–27. [DOI] [PubMed]

42.

FitzGerald JM, Bleecker ER, Nair P, Korn S, Ohta K, Lommatzsch M, et al.; CALIMA study investigators. Benralizumab, an anti-interleukin-5 receptor α monoclonal antibody, as add-on treatment for patients with severe, uncontrolled, eosinophilic asthma (CALIMA): a randomised, doubleblind, placebocontrolled phase 3 trial. Lancet. 2016;388:2128–41. [DOI] [PubMed]

43.

Nair P, Wenzel S, Rabe KF, Bourdin A, Lugogo NL, Kuna P, et al.; ZONDA Trial Investigators. Oral glucocorticoid–sparing effect of benralizumab in severe asthma. N Engl J Med. 2017;376:2448–58. [DOI] [PubMed]

44.

Takenaka K, Minami T, Yoshihashi Y, Hirata S, Kimura Y, Kono H. Decrease in MPO-ANCA after administration of benralizumab in eosinophilic granulomatosis with polyangiitis. Allergol Int. 2019;68:539–40. [DOI] [PubMed]

45.

Coppola A, Flores KR, De Filippis F. Rapid onset of effect of benralizumab on respiratory symptoms in a patient with eosinophilic granulomatosis with polyangiitis. Respir Med Case Rep. 2020;30:101050. [DOI] [PubMed] [PMC]

46.

Colantuono S, Pellicano C, Leodori G, Cilia F, Francone M, Visentini M. Early benralizumab for eosinophilic myocarditis in eosinophilic granulomatosis with polyangiitis. Allergol Int. 2020;69:483–4. [DOI] [PubMed]

47.

Lee Y, Hojjati M. Benralizumab as a potential adjunctive therapy in eosinophilic granulomatosis with polyangiitis. J Clin Rheumatol. 2021;27:S401–2. [DOI] [PubMed]

48.

Carrillo-Martin I, Abril A, Donaldson AM, Gonzalez-Estrada A. An alternative approach against eosinophils for the treatment of eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol Pract. 2020;8:2079–80. [DOI] [PubMed]

49.

Chica-Guzmán MV, Morillo-Guerrero R, Carrón-Herrero A, González-de-Olano D, Almonacid-Sánchez C. Eosinophilic granulomatosis with polyangiitis successfully treated with benralizumab. Ann Allergy Asthma Immunol. 2020;125:228–30. [DOI] [PubMed]

50.

Martínez-Rivera C, Garcia-Olivé I, Urrutia-Royo B, Basagaña-Torrento M, Rosell A, Abad J. Rapid effect of benralizumab in exacerbation of severe eosinophilic asthma associated with eosinophilic granulomatosis with polyangiitis. BMC Pulm Med. 2021;21:35. [DOI] [PubMed] [PMC]

51.

Bormioli S, Vultaggio A, Nencini F, Comin CE, Bercich L, Bezzi M, et al. Benralizumab: resolution of eosinophilic pulmonary vasculitis in a patient with EGPA. J Investig Allergol Clin Immunol. 2021;31:519–21. [DOI] [PubMed]

52.

Kolios AGA, Lutterotti A, Kulcsar Z, Renner T, Rudiger A, Nilsson J. Benralizumab in eosinophilic granulomatosis with polyangiitis complicated by Staphylococcus aureus sepsis. Clin Immunol. 2021;222:108574. [DOI] [PubMed]

53.

Lim AKH, Antony A, Gingold M, Simpson I, Looi WF, MacDonald MI. Emergence of extrathoracic manifestations of eosinophilic granulomatosis with polyangiitis during benralizumab treatment. Rheumatol Adv Pract. 2021;5:rkab033. [DOI] [PubMed] [PMC]

54.

Miyata Y, Inoue H, Homma T, Tanaka A, Sagara H. Efficacy of benralizumab and clinical course of IgG4 in eosinophilic granulomatosis with polyangiitis. J Investig Allergol Clin Immunol. 2021;31:346–8. [DOI] [PubMed]

55.

Koga Y, Aoki-Saito H, Kamide Y, Sato M, Tsurumaki H, Yatomi M, et al. Perspectives on the efficacy of benralizumab for treatment of eosinophilic granulomatosis with polyangiitis. Front Pharmacol. 2022;13:865318. [DOI] [PubMed] [PMC]

56.

Padoan R, Chieco Bianchi F, Marchi MR, Cazzador D, Felicetti M, Emanuelli E, et al. Benralizumab as a glucocorticoid-sparing treatment option for severe asthma in eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol Pract. 2020;8:3225–7.E2. [DOI] [PubMed]

57.

Nanzer AM, Dhariwal J, Kavanagh J, Hearn A, Fernandes M, Thomson L, et al. Steroid-sparing effects of benralizumab in patients with eosinophilic granulomatosis with polyangiitis. ERJ Open Res. 2020;6:00451-2020. [DOI] [PubMed] [PMC]

58.

Guntur VP, Manka LA, Denson JL, Dunn RM, Dollin YT, Gill M, et al. Benralizumab as a steroid-sparing treatment option in eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol Pract. 2021;9:1186–93.E1. [DOI] [PubMed]

59.

Benralizumab in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) study (BITE) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 30]. Available from: https://clinicaltrials.gov/ct2/show/NCT03010436?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=14&rank=23

60.

Efficacy and safety of benralizumab in EGPA compared to mepolizumab. (MANDARA) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Oct 30]. Available from: https://clinicaltrials.gov/ct2/show/NCT04157348?cond=EGPA++Eosinophilic+Granulomatosis+with+Polyangiitis&draw=17&rank=9

61.

Castro M, Zangrilli J, Wechsler ME, Bateman ED, Brusselle GG, Bardin P, et al. Reslizumab for inadequately controlled asthma with elevated blood eosinophil counts: results from two multicentre, parallel, double-blind, randomised, placebo-controlled, phase 3 trials. Lancet Respir Med. 2015;3:355–66. [DOI] [PubMed]

62.

Kent BD, d’Ancona G, Fernandes M, Green L, Roxas C, Thomson L, et al. Oral corticosteroid-sparing effects of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis. ERJ Open Res. 2020;6:00311-2019. [DOI] [PubMed] [PMC]

63.

Manka LA, Guntur VP, Denson JL, Dunn RM, Dollin YT, Strand MJ, et al. Efficacy and safety of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis. Ann Allergy Asthma Immunol. 2021;126:696–701.E1. [DOI] [PubMed]

64.

Navinés-Ferrer A, Serrano-Candelas E, Molina-Molina GJ, Martín M. IgE-related chronic diseases and anti-IgE-based treatments. J Immunol Res. 2016;2016:8163803. [DOI] [PubMed] [PMC]

65.

Basta F, Mazzuca C, Nucera E, Schiavino D, Afeltra A, Antonelli Incalzi R. Omalizumab in eosinophilic granulomatosis with polyangiitis: friend or foe? A systematic literature review. Clin Exp Rheumatol. 2020;38:214–20. [PubMed]

66.

Giavina-Bianchi P, Giavina-Bianchi M, Agondi R, Kalil J. Three months’ administration of anti-IgE to a patient with Churg-Strauss syndrome. J Allergy Clin Immunol. 2007;119:1279. [DOI] [PubMed]

67.

Pabst S, Tiyerili V, Grohé C. Apparent response to anti-IgE therapy in two patients with refractory “forme fruste” of Churg–Strauss syndrome. Thorax. 2008;63:747–8. [DOI] [PubMed]

68.

Lau EMT, Cooper W, Bye PT, Yan K. Difficult asthma and Churg-Strauss-like syndrome: a cautionary tale. Respirology. 2011;16:180–1. [DOI] [PubMed]

69.

Graziani A, Quercia O, Girelli F, Martelli A, Mirici Cappa F, Stefanini GF. Omalizumab treatment in patient with severe asthma and eosinophilic granulomatosis with polyangiitis. A case report. Eur Ann Allergy Clin Immunol. 2014;46:226–8. [PubMed]

70.

Caruso C, Gencarelli G, Gaeta F, Valluzzi RL, Rumi G, Romano A. Efficacy of omalizumab treatment in a man with occupational asthma and eosinophilic granulomatosis with polyangioitis. Ann Allergy Asthma Immunol. 2018;120:209–11. [DOI] [PubMed]

71.

Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, Bonniaud P, et al.; French Vasculitis Study Group. Anti-IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss): data on seventeen patients. Arthritis Rheumatol. 2016;68:2274–82. [DOI] [PubMed]

72.

Celebi Sozener Z, Gorgulu B, Mungan D, Sin BA, Misirligil Z, Aydin O, et al. Omalizumab in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA): single-center experience in 18 cases. World Allergy Organ J. 2018;11:39. [DOI] [PubMed] [PMC]

73.

Puéchal X, Rivereau P, Vinchon F. Churg-Strauss syndrome associated with omalizumab. Eur J Intern Med. 2008;19:364–6. [DOI] [PubMed]

74.

Bargagli E, Madioni C, Olivieri C, Penza F, Rottoli P. Churg-Strauss vasculitis in a patient treated with omalizumab. J Asthma. 2008;45:115–6. [DOI] [PubMed]

75.

Ruppert AM, Averous G, Stanciu D, Deroide N, Riehm S, Poindron V, et al. Development of Churg-Strauss syndrome with controlled asthma during omalizumab treatment. J Allergy Clin Immunol. 2008;121:253–4. [DOI] [PubMed]

76.

Cazzola M, Mura M, Segreti A, Mattei MA, Rogliani P. Eosinophilic pneumonia in an asthmatic patient treated with omalizumab therapy: forme-fruste of Churg-Strauss syndrome? Allergy. 2009;64:1389–90. [DOI] [PubMed]

77.

Cisneros C, Segrelles G, Herráez L, Gonzalez A, Girón R. Churg-Strauss syndrome in a patient treated with omalizumab. J Investig Allergol Clin Immunol. 2013;23:504–21. [PubMed]

78.

Charles P, Néel A, Tieulié N, Hot A, Pugnet G, Decaux O, et al.; French Vasculitis Study Group. Rituximab for induction and maintenance treatment of ANCA-associated vasculitides: a multicentre retrospective study on 80 patients. Rheumatology (Oxford). 2014;53:532–9. [DOI] [PubMed]

79.

Puéchal X. Therapeutic immunomodulation in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Joint Bone Spine. 2016;83:7–10. [DOI] [PubMed]

80.

Jones RB, Tervaert JWC, Hauser T, Luqmani R, Morgan MD, Peh CA, et al.; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363:211–20. [DOI] [PubMed]

81.

Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al.; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANC-associated vasculitis. N Engl J Med. 2010;363:221–32. [DOI] [PubMed] [PMC]

82.

Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, et al.; RAVE-ITN Research Group. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369:417–27. [DOI] [PubMed] [PMC]

83.

Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Ann Rheum Dis. 2016;75:396–401. [DOI] [PubMed]

84.

Novikov P, Moiseev S, Smitienko I, Zagvozdkina E. Rituximab as induction therapy in relapsing eosinophilic granulomatosis with polyangiitis: a report of 6 cases. Joint Bone Spine. 2016;83:81–4. [DOI] [PubMed]

85.

Thiel J, Hässler F, Salzer U, Voll RE, Venhoff N. Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Arthritis Res Ther. 2013;15:R133. [DOI] [PubMed] [PMC]

86.

Emmi G, Rossi GM, Urban ML, Silvestri E, Prisco D, Goldoni M, et al. Scheduled rituximab maintenance reduces relapse rate in eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2018;77:952–4. [DOI] [PubMed]

87.

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. 2019;5:e000905. [DOI] [PubMed] [PMC]

88.

Casal Moura M, Berti A, Keogh KA, Volcheck GW, Specks U, Baqir M. Asthma control in eosinophilic granulomatosis with polyangiitis treated with rituximab. Clin Rheumatol. 2020;39:1581–90. [DOI] [PubMed]

89.

Cartin-Ceba R, Keogh KA, Specks U, Sethi S, Fervenza FC. Rituximab for the treatment of Churg-Strauss syndrome with renal involvement. Nephrol Dial Transplant. 2011;26:2865–71. [DOI] [PubMed] [PMC]

90.

Rituximab in eosinophilic granulomatosis with polyangiitis (REOVAS) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Nov 06]. Available from: https://clinicaltrials.gov/ct2/show/NCT02807103

91.

Maintenance of remission with rituximab versus azathioprine for newly-diagnosed or relapsing eosinophilic granulomatosis with polyangiitis (MAINRITSEG) [Internet]. Bethesda: National Library of Medicine; c2022 [cited 2022 Nov 06]. Available from: https://clinicaltrials.gov/ct2/show/NCT03164473

92.

Wu EY, McInnis EA, Boyer-Suavet S, Mendoza CE, Aybar LT, Kennedy KB, et al. Measuring circulating complement activation products in myeloperoxidase- and proteinase 3-antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2019;71:1894–903. [DOI] [PubMed] [PMC]

93.

Jennette JC, Xiao H, Falk R, Gasim AMH. Experimental models of vasculitis and glomerulonephritis induced by antineutrophil cytoplasmic autoantibodies. Contrib Nephrol. 2011;169:211–20. [DOI] [PubMed] [PMC]

94.

Xiao H, Schreiber A, Heeringa P, Falk RJ, Jennette JC. Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies. Am J Pathol. 2007;170:52–64. [DOI] [PubMed] [PMC]

95.

Schreiber A, Xiao H, Jennette JC, Schneider W, Luft FC, Kettritz R. C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis. J Am Soc Nephrol. 2009;20:289–98. [DOI] [PubMed] [PMC]

96.

Xiao H, Dairaghi DJ, Powers JP, Ertl LS, Baumgart T, Wang Y. C5a receptor (CD88) blockade protects against MPO-ANCA GN. J Am Soc Nephrol. 2014;25:225–31. [DOI] [PubMed] [PMC]

97.

Tesar V, Hruskova Z. Complement inhibition in ANCA-associated vasculitis. Front Immunol. 2022;13:888816. [DOI] [PubMed] [PMC]