Exploration of Immunology

Table 3. There are several ongoing clinical trials and funding for the treatment of EGPA.

S. No.	NCT numbers	Title	Status	Interventions	Phase	Populations 18 years to 50 years (adult, older adult)	Funding	Result
1	NCT06230354	Explore the Efficacy and Mechanism of Action of Tezepelumab in Eosinophilic Granulomatosis with Polyangiitis (RACEMATE)	Enrolling by invitation	Tezepelumab, placebo	Phase 2	62	Imperial College London	Publicly not available
2	NCT05979051	A Study to Evaluate the Efficacy and Safety of SHR-1703 in Subjects With EGPA	Recruiting	SHR-1703, SHR-1703 Placebo	Phase 2–3	112	Guangdong Hengrui Pharmaceutical Co., Ltd	The result is not publicly available
3	NCT05263934	Efficacy and Safety of Depemokimab Compared with Mepolizumab in Adults with Relapsing or Refractory EGPA (OCEAN)	Recruiting	Biological: Depemokimab Biological: Mepolizumab Placebo matching mepolizumab, depemokimab	Phase 3	160	GlaxoSmithKline	Publicly not available
4	NCT02807103	Rituximab in Eosinophilic Granulomatosis with Polyangiitis (REOVAS)	Completed	Rituximab Placebo-rituximab Cyclophosphamide Placebo-cyclophosphamide	Phase 3	107	Assistance Publique—Hôpitaux de Paris	Rituximab has shown a significant reduction in symptoms of EGPA compared to cyclophosphamide and glucocorticoids
5	NCT04551989	Mepolizumab Long-term Study to Assess Real World Safety and Effectiveness of EGPA In Japan (MARS)	Completed	-	Observational	120	GlaxoSmithKline	Mepolizumab is effective in managing EGPA symptoms and reducing corticosteroid dependency

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EGPA: eosinophilic granulomatosis with polyangiitis.

Declarations

Acknowledgments

We extend our heartfelt gratitude to the management of Rungta International Skills University, Bhilai, for their unwavering support and encouragement throughout this work. Their dedication to fostering a research-conducive environment has been instrumental in completing this review.

Author contributions

PK: Writing—original draft, Conceptualization. AG: Methodology, Resources, Writing—original draft. SKG: Writing—review & editing, Project administration, Supervision, Validation. TMJ and DKM: Data curation. 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

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Consent to publication

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Availability of data and materials

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Funding

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References

Mattioli I, Urban ML, Padoan R, Mohammad AJ, Salvarani C, Baldini C, et al.; Eurpean EGPA Study Group. Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study. J Autoimmun. 2025;153:103398. [DOI] [PubMed]

Sato M, Nagai K, Sato T, Yoshimoto R, Shibano Y, Shibahara M, et al. Aberrant oxidative modifications of neutrophil myeloperoxidase in anti-neutrophil cytoplasmic antibody-associated vasculitis. J Proteomics. 2025;315:105412. [DOI] [PubMed]

Rupani H, Bansal AT, Sergejeva S, Nanzer A, Caminati M, Kopac P, et al. Newly diagnosed eosinophilic granulomatosis with polyangiitis in patients on biologics for severe asthma: A multicenter case series. J Allergy Clin Immunol Pract. 2025;13:1859–62.e3. [DOI] [PubMed]

Gruden E, Kienzl M, Danner L, Kaspret DM, Pammer A, Ristic D, et al. The Endocannabinoid System Drives Eosinophil Infiltration During Eosinophilic Esophagitis. Cell Mol Gastroenterol Hepatol. 2025;19:101515. [DOI] [PubMed] [PMC]

Silver J, Deb A, Packnett E, McMorrow D, Morrow C, Bogart M. Characteristics and Disease Burden of Patients With Eosinophilic Granulomatosis With Polyangiitis Initiating Mepolizumab in the United States. J Clin Rheumatol. 2023;29:381–7. [DOI] [PubMed] [PMC]

White J, Dubey S. Eosinophilic granulomatosis with polyangiitis: A review. Autoimmun Rev. 2023;22:103219. [DOI] [PubMed]

WECHSLER ME, AKUTHOTA P, JAYNE D, HELLMICH B, KHOURY P, PRICE R, et al. Mepolizumab Reduced Steroid Burden for Patients With Eosinophilic Granulomatosis With Polyangiitis With and Without a Vasculitic Phenotype. Chest. 2023;164:A46–9.

AlBloushi S, Al-Ahmad M. Exploring the immunopathology of type 2 inflammatory airway diseases. Front Immunol. 2024;15:1285598. [DOI] [PubMed] [PMC]

Jakes RW, Kwon N, Nordstrom B, Goulding R, Fahrbach K, Tarpey J, et al. Burden of illness associated with eosinophilic granulomatosis with polyangiitis: a systematic literature review and meta-analysis. Clin Rheumatol. 2021;40:4829–36. [DOI] [PubMed] [PMC]

10.

Mouthon L, Dunogue B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun. 2014;48–49:99–103. [DOI] [PubMed]

11.

Bloom JL, Langford CA, Wechsler ME. Therapeutic Advances in Eosinophilic Granulomatosis with Polyangiitis. Rheum Dis Clin North Am. 2023;49:563–84. [DOI] [PubMed]

12.

Solans-Laqué R, Rúa-Figueroa I, Aparicio MB, Moguel IG, Blanco R, Grimaldi FP, et al. Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA). Eur J Intern Med. 2024;128:45–52. [DOI] [PubMed]

13.

Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. [DOI] [PubMed] [PMC]

14.

Akhouri S, House SA. Allergic Rhinitis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. [PubMed]

15.

Carnicelli G, Sernicola A, Gomes V, Cundari G, Trasarti S, Priori R, et al. Multiple Cutaneous Manifestations in ANCA-Positive Eosinophilic Granulomatosis with Polyangiitis before and after Biologic Therapy: Clinical and Histopathologic Characterization of a Paradigmatic Case. J Clin Med. 2022;11:7429. [DOI] [PubMed] [PMC]

16.

Gupta SK, Bodakhe SH. Development of pharmacological screening method for evaluation of effect of drug on elevated pulse pressure and arterial stiffness. J Pharmacol Toxicol Methods. 2018;91:59–65. [DOI]

17.

Zaffar S, Saha S, Agrawal T, Gupta SK, Gulati P, Paul D, et al. Improved Diabetic Wound Healing via Flower Extracellular Vesicles and Carbon-Dot-Infused Alginate-Polyethylenimine Antibacterial Hydrogel. ACS Biomater Sci Eng. 2025;11:4219–30. [DOI]

18.

Stanton M, Tiwari V. Polyarteritis Nodosa. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. [PubMed]

19.

Alam V, Nanzer AM. Eosinophilic granulomatosis with polyangiitis: case report and literature review. Breathe (Sheff). 2022;18:220170. [DOI] [PubMed] [PMC]

20.

Spray J, Hunleth J, Ruiz S, Maki J, Fedele DA, Prabhakaran S, et al. How do embodied experiences of asthma influence caregiver conceptual models? Soc Sci Med. 2022;294:114706. [DOI] [PubMed] [PMC]

21.

Gomes L, Santos SD, Pereira S, Rua J, Fortuna J. Sinusitis and Late-Onset Asthma: A Red Flag of Eosinophilic Granulomatosis With Polyangiitis. Cureus. 2023;15:e35512. [DOI] [PubMed] [PMC]

22.

Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023;19:378–93. [DOI] [PubMed]

23.

Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, Lazor R, et al.; the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P). Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J. 2016;48:1429–41. [DOI] [PubMed]

24.

Morita TCAB, Criado PR, Criado RFJ, Trés GFS, Sotto MN. Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis–Part II. An Bras Dermatol. 2020;95:493–507. [DOI] [PubMed] [PMC]

25.

Koike H, Nishi R, Ohyama K, Morozumi S, Kawagashira Y, Furukawa S, et al. ANCA-Associated Vasculitic Neuropathies: A Review. Neurol Ther. 2022;11:21–38. [DOI] [PubMed] [PMC]

26.

Bolotin D, O’Brien C, Ranganath VK, Kermani TA. ANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study. Semin Arthritis Rheum. 2025;71:152648. [DOI] [PubMed]

27.

Kim M, Lee S, Song W, Lee S, Kang H, Kim S, et al. Clinical Features And Prognostic Factors Of Churg-strauss Syndrome. J Allergy Clin Immunol. 2012;129:AB214.

28.

Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity. 2005;38:93–103. [DOI] [PubMed]

29.

Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17:603–6. [DOI] [PubMed] [PMC]

30.

Memon RJ, Savliwala MN. Eosinophilic Gastroenteritis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. [PubMed]

31.

Samson M, Puéchal X, Devilliers H, Ribi C, Cohen P, Stern M, et al.; French Vasculitis Study Group. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013;43:60–9. [DOI] [PubMed]

32.

Takahashi N, Ikoma T, Sakamoto A, Suwa K, Fujihiro M, Shimoyama K, et al. Ventricular Thrombus Formation Caused by Subendomyocardial Inflammation in Eosinophilic Granulomatosis With Polyangiitis. JACC Case Rep. 2024;29:102321. [DOI] [PubMed] [PMC]

33.

Furuta S, Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis. Allergol Int. 2019;68:430–6. [DOI] [PubMed]

34.

Meshram HK, Gupta SK, Gupta A, Nagori K, Ajazuddin. Next-generation CRISPR gene editing tools in the precision treatment of Alzheimer’s and Parkinson’s disease. Ageing Res Rev. 2025;111:102851. [DOI]

35.

Pakbaz M, Pakbaz M. Cardiac involvement in eosinophilic granulomatosis with polyangiitis: A meta-analysis of 62 case reports. J Tehran Heart Cent. 2020;15:18–26. [PubMed] [PMC]

36.

Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic Manifestations of Systemic Necrotizing Vasculitides at Diagnosis: A Retrospective Study of 1286 Patients and Review of the Literature. Semin Arthritis Rheum. 2013;42:507–14. [DOI] [PubMed]

37.

Ramirez GA, Yacoub MR, Ripa M, Mannina D, Cariddi A, Saporiti N, et al. Eosinophils from Physiology to Disease: A Comprehensive Review. Biomed Res Int. 2018;2018:9095275. [DOI] [PubMed] [PMC]

38.

Chakraborty RK, Rout P. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome). In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. [PubMed]

39.

Trivioli G, Terrier B, Vaglio A. Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. Rheumatology (Oxford). 2020;59:iii84–94. [DOI] [PubMed]

40.

Khoury P, Akuthota P, Kwon N, Steinfeld J, Roufosse F. HES and EGPA: Two Sides of the Same Coin. Mayo Clin Proc. 2023;98:1054–70. [DOI] [PubMed] [PMC]

41.

Garrido CV, Mukherjee M, Svenningsen S, Nair P. Eosinophil-mucus interplay in severe asthma: Implications for treatment with biologicals. Allergol Int. 2024;73:351–61. [DOI] [PubMed]

42.

Asano K, Suzuki Y, Tanaka J, Kobayashi K, Kamide Y. Treatments of refractory eosinophilic lung diseases with biologics. Allergol Int. 2023;72:31–40. [DOI] [PubMed]

43.

Pitlick MM, Li JT, Pongdee T. Current and emerging biologic therapies targeting eosinophilic disorders. World Allergy Organ J. 2022;15:100676. [DOI] [PubMed] [PMC]

44.

Farrah TE, Melville V, Czopek A, Fok H, Bruce L, Mills NL, et al. Arterial stiffness, endothelial dysfunction and impaired fibrinolysis are pathogenic mechanisms contributing to cardiovascular risk in ANCA-associated vasculitis. Kidney Int. 2022;102:1115–26. [DOI] [PubMed]

45.

Chehade M, Falk GW, Aceves S, Lee JK, Mehta V, Leung J, et al. Examining the Role of Type 2 Inflammation in Eosinophilic Esophagitis. Gastro Hep Adv. 2022;1:720–32. [DOI] [PubMed] [PMC]

46.

Jariwala M, Laxer RM. Childhood GPA, EGPA, and MPA. Clin Immunol. 2020;211:108325. [DOI] [PubMed]

47.

Scott J, Hartnett J, Mockler D, Little MA. Environmental risk factors associated with ANCA associated vasculitis: A systematic mapping review. Autoimmun Rev. 2020;19:102660. [DOI] [PubMed]

48.

Kallenberg CG, Stegeman CA, Abdulahad WH, Heeringa P. Pathogenesis of ANCA-associated vasculitis: new possibilities for intervention. Am J Kidney Dis. 2013;62:1176–87. [DOI] [PubMed]

49.

Izquierdo-Domínguez A, Cordero Castillo A, Alobid I, Mullol J. Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis. Sinusitis. 2015;1:24–43. [DOI]

50.

Valent P, Klion AD, Roufosse F, Simon D, Metzgeroth G, Leiferman KM, et al. Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes. Allergy. 2023;78:47–59. [DOI] [PubMed] [PMC]

51.

Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy. 2013;68:261–73. [DOI] [PubMed]

52.

Fijolek J, Radzikowska E. Eosinophilic granulomatosis with polyangiitis–Advances in pathogenesis, diagnosis, and treatment. Front Med (Lausanne). 2023;10:1145257. [DOI] [PubMed] [PMC]

53.

Parola M, Pinzani M. Liver fibrosis in NAFLD/NASH: from pathophysiology towards diagnostic and therapeutic strategies. Mol Aspects Med. 2024;95:101231. [DOI] [PubMed]

54.

Springer JM, Kalot MA, Husainat NM, Byram KW, Dua AB, James KE, et al. Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis of Test Accuracy and Benefits and Harms of Common Treatments. ACR Open Rheumatol. 2021;3:101–10. [DOI] [PubMed] [PMC]

55.

Del Pozo V, Bobolea I, Rial MJ, Espigol-Frigolé G, Solans Laqué R, Hernández-Rivas JM, et al. Expert consensus on the use of systemic glucocorticoids for managing eosinophil-related diseases. Front Immunol. 2024;14:1310211. [DOI] [PubMed] [PMC]

56.

Gezgin Yildirim D, Orulluoglu EY, Yildiz C, Acari C, Dundar HA, Akaci O, et al. Cyclophosphamide treatment with a comparison in both pediatric rheumatology and pediatric nephrology practices. Pediatr Rheumatol. 2025;23:24. [DOI]

57.

Hamour S, Salama AD, Pusey CD. Management of ANCA-associated vasculitis: Current trends and future prospects. Ther Clin Risk Manag. 2010;6:253–64. [DOI] [PubMed] [PMC]

58.

Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75:1583–94. [DOI] [PubMed]

59.

Tzortzatou-Stathopoulou F, Zavitsa-Pateli H, Mousatos G, Prodromou N, Kairis M, Pappas J. Action of Intermediate Doses of Methotrexate on Dihydrofolate Reductase in Malignant Diseases. Pediatr Hematol Oncol. 1987;4:43–53. [DOI] [PubMed]

60.

Goldszer RC, Sweet J, Cotran RS. Focal segmental glomerulosclerosis. Annu Rev Med. 1984;35:429–49. [DOI] [PubMed]

61.

Friedman AB, Sparrow MP, Gibson PR. Thiopurines: Azathioprine, Mercaptopurine, and Thioguanine. Compendium of Inflammatory Diseases. Basel: Springer Basel; 2016. pp. 1–12. [DOI]

62.

Detoraki A, Tremante E, Poto R, Morelli E, Quaremba G, Granata F, et al. Real-life evidence of low-dose mepolizumab efficacy in EGPA: a case series. Respir Res. 2021;22:185. [DOI] [PubMed] [PMC]

63.

Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376:1921–32. [DOI] [PubMed] [PMC]

64.

Csernok E, Mahrhold J, Hellmich B. Anti-neutrophil cytoplasm antibodies (ANCA): Recent methodological advances-Lead to new consensus recommendations for ANCA detection. J Immunol Methods. 2018;456:1–6. [DOI] [PubMed]

65.

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

66.

Wagener AH, de Nijs SB, Lutter R, Sousa AR, Weersink EJ, Bel EH, et al. External validation of blood eosinophils, FE_NO and serum periostin as surrogates for sputum eosinophils in asthma. Thorax. 2015;70:115–20. [DOI] [PubMed]

67.

Desaintjean C, Ahmad K, Traclet J, Gerfaud-Valentin M, Durel CA, Glerant JC, et al. Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis. Front Med (Lausanne). 2024;11:1341310. [DOI] [PubMed] [PMC]

68.

Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26:545–53. [DOI] [PubMed]

69.

Sinha S, Gupta SK, Sahu P, Gupta A, Ajazuddin. Capsaicin: Unveiling its Therapeutic Potential and Pharmacological Actions. Curr Bioact Compd. 2025:21. [DOI]

70.

Rajgopal B, Gupta SK, Deshmukh R, Gupta A, Patel A, Sakure K, et al. Emerging Trends in Hydrogels for the Treatment of Vaginal Candidiasis: A Comprehensive Review. Recent Adv Antiinfect Drug Discov. 2025;20:168–82. [DOI] [PubMed]

71.

Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73:1366–83. [DOI] [PubMed] [PMC]

72.

Wallace ZS, Miloslavsky EM. Management of ANCA associated vasculitis. BMJ. 2020;368:m421. [DOI] [PubMed]

73.

Lagan J, Naish JH, Fortune C, Bradley J, Clark D, Niven R, et al. Myocardial involvement in eosinophilic granulomatosis with polyangiitis evaluated with cardiopulmonary magnetic resonance. Int J Cardiovasc Imaging. 2021;37:1371–81. [DOI] [PubMed] [PMC]

74.

Harper L, Morgan MD, Walsh M, Hoglund P, Westman K, Flossmann O, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71:955–60. [DOI] [PubMed]

75.

de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, et al.; EUVAS (European Vasculitis Study Group). Pulse Versus Daily Oral Cyclophosphamide for Induction of Remission in Antineutrophil Cytoplasmic Antibody—Associated Vasculitis: A Randomized Trial. Ann Intern Med. 2009;150:670–80. [DOI] [PubMed]

76.

Pagnoux C, Quéméneur T, Ninet J, Diot E, Kyndt X, Wazières Bd, et al.; French Vasculitis Study Group. Treatment of Systemic Necrotizing Vasculitides in Patients Aged Sixty-Five Years or Older: Results of a Multicenter, Open-Label, Randomized Controlled Trial of Corticosteroid and Cyclophosphamide–Based Induction Therapy. Arthritis Rheumatol. 2015;67:1117–27. [DOI] [PubMed]

77.

Cohen P, Pagnoux C, Mahr A, Arène JP, Mouthon L, Le Guern V, et al.; French Vasculitis Study Group. 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. 2007;57:686–93. [DOI] [PubMed]

78.

Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al.; European Vasculitis Study Group (EUVAS). Mycophenolate Mofetil vs Azathioprine for Remission Maintenance in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A Randomized Controlled Trial. JAMA. 2010;304:2381–8. [DOI] [PubMed]

79.

Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix J, et al.; French Vasculitis Study Group. Azathioprine or Methotrexate Maintenance for ANCA-Associated Vasculitis. N Engl J Med. 2008;359:2790–803. [DOI] [PubMed]

80.

Puéchal X, Pagnoux C, Baron G, Quémeneur T, Néel A, Agard C, et al.; French Vasculitis Study Group. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial. Arthritis Rheumatol. 2017;69:2175–86. [DOI] [PubMed]

81.

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]

82.

Tsurikisawa N, Oshikata C, Watanabe M, Fukuda N, Yamaguchi T, Kiyohara H, et al. Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab. Int Arch Allergy Immunol. 2021;182:744–56. [DOI] [PubMed]

83.

Walsh M, Casian A, Flossmann O, Westman K, Höglund P, Pusey C, et al.; European Vasculitis Study Group (EUVAS). Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 2013;84:397–402. [DOI] [PubMed]

84.

Uzzo M, Regola F, Trezzi B, Toniati P, Franceschini F, Sinico RA. Novel Targets for Drug Use in Eosinophilic Granulomatosis With Polyangiitis. Front Med (Lausanne). 2021;8:754434. [DOI] [PubMed] [PMC]

85.

Puan Y, Ong KY, Tiew PY, Wen Chen GX, Teo NWY, Low AHL, et al. Characteristics of Severe Asthma Clinic Patients With Eosinophilic Granulomatosis With Polyangiitis. J Allergy Clin Immunol Pract. 2025;13:361–8.e2. [DOI] [PubMed]

86.

Carrón-Herrero A, Pelaia C, Paoletti G. New therapeutic approaches with biological drugs for eosinophilic granulomatosis with polyangiitis. Explor Asthma Allergy. 2023;1:31–48. [DOI]

87.

Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G. Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome. Ann Rheum Dis. 2004;63:1649–54. [DOI] [PubMed] [PMC]

88.

Jones RB, Tervaert JW, 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]

89.

Leget GA, Czuczman MS. Use of rituximab, the new FDA-approved antibody. Curr Opin Oncol. 1998;10:548–51. [DOI] [PubMed]

90.

Looney RJ. B Cell-Targeted Therapy for Rheumatoid Arthritis: An Update on the Evidence. Drugs. 2006;66:625–39. [DOI] [PubMed]

91.

Alberici F, Jayne DR. Impact of rituximab trials on the treatment of ANCA-associated vasculitis. Nephrol Dial Transplant. 2014;29:1151–9. [DOI] [PubMed]

92.

Akiyama M, Kaneko Y, Takeuchi T. Eosinophilic granulomatosis with polyangiitis can manifest lacrimal and salivary glands swelling by granulomatous inflammation: a potential mimicker of IgG4-related disease. Ann Rheum Dis. 2022;81:e120. [DOI] [PubMed]

93.

Vaglio A, Strehl JD, Manger B, Maritati F, Alberici F, Beyer C, et al. IgG4 immune response in Churg-Strauss syndrome. Ann Rheum Dis. 2012;71:390–3. [DOI] [PubMed]

94.

Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. Decreases in the Numbers of Peripheral Blood Regulatory T Cells, and Increases in the Levels of Memory and Activated B Cells, in Patients with Active Eosinophilic Granulomatosis and Polyangiitis. J Clin Immunol. 2013;33:965–76. [DOI] [PubMed]

95.

Popa ER, Stegeman CA, Bos NA, Kallenberg CG, Tervaert JW. Differential B- and T-cell activation in Wegener’s granulomatosis. J Allergy Clin Immunol. 1999;103:885–94. [DOI] [PubMed]

96.

Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al.; French Vasculitis Study Group. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771–80. [DOI] [PubMed]

97.

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]

98.

Wilde B, van Paassen P, Witzke O, Tervaert JWC. New pathophysiological insights and treatment of ANCA-associated vasculitis. Kidney Int. 2011;79:599–612. [DOI] [PubMed]

99.

Luther SA, Cyster JG. Chemokines as regulators of T cell differentiation. Nat Immunol. 2001;2:102–7. [DOI] [PubMed]

100.

Principe S, Porsbjerg C, Bolm Ditlev S, Kjaersgaard Klein D, Golebski K, Dyhre-Petersen N, et al. Treating severe asthma: Targeting the IL-5 pathway. Clin Exp Allergy. 2021;51:992–1005. [DOI] [PubMed] [PMC]

101.

Menzella F, Ruggiero P, Ghidoni G, Fontana M, Bagnasco D, Livrieri F, et al. Anti-IL5 Therapies for Severe Eosinophilic Asthma: Literature Review and Practical Insights. J Asthma Allergy. 2020;13:301–13. [DOI] [PubMed] [PMC]

102.

Fagni F, Bello F, Emmi G. Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology. Front Med (Lausanne). 2021;8:627776. [DOI] [PubMed] [PMC]

103.

Faverio P, Bonaiti G, Bini F, Vaghi A, Pesci A. Mepolizumab as the first targeted treatment for eosinophilic granulomatosis with polyangiitis: a review of current evidence and potential place in therapy. Ther Clin Risk Manag. 2018;14:2385–96. [DOI] [PubMed] [PMC]

104.

Daboussi S, Essebaa S, Mhamdi S, Aichaouia C, Hela G, Ayadi A, et al. Case Report: Mepolizumab in the treatment of idiopathic chronic eosinophilic pneumonia. F1000Res. 2023;12:337. [DOI] [PubMed] [PMC]

105.

Bölke G, Tong X, Zuberbier T, Bousquet J, Bergmann KC. Extension of mepolizumab injection intervals as potential of saving costs in well controlled patients with severe eosinophilic asthma. World Allergy Organ J. 2022;15:100703. [DOI] [PubMed] [PMC]

106.

Vultaggio A, Nencini F, Bormioli S, Vivarelli E, Dies L, Rossi O, et al. Low-Dose Mepolizumab Effectiveness in Patients Suffering From Eosinophilic Granulomatosis With Polyangiitis. Allergy Asthma Immunol Res. 2020;12:885–93. [DOI] [PubMed] [PMC]

107.

Ryser FS, Yalamanoglu A, Valaperti A, Brühlmann C, Mauthe T, Traidl S, et al. Dupilumab-induced eosinophilia in patients with diffuse type 2 chronic rhinosinusitis. Allergy. 2023;78:2712–23. [DOI] [PubMed]

108.

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]

109.

Menzella F, Just J, Sauerbeck IS, Mailaender C, Saccheri F, Thonnelier C, et al. Omalizumab for the treatment of patients with severe allergic asthma with immunoglobulin E levels above >1500 IU/mL. World Allergy Organ J. 2023;16:100787. [DOI] [PubMed] [PMC]

110.

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83:30–47. [DOI] [PubMed]

111.

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]

112.

Brannick S, McDonald M, Greally P, Elnazir B, Ahmareen O. Omalizumab for the treatment of severe allergic asthma in children: A tale of two. Clin Case Rep. 2022;10:e6255. [DOI] [PubMed] [PMC]

113.

Novacic D, Uldrick T, Dulau-Florea A, Howe CE, Lee CR, Kong HH, et al. Calciphylaxis in POEMS syndrome: Case report. Rare. 2024;2:100019. [DOI] [PubMed] [PMC]

114.

Kouverianos I, Angelopoulos A, Daoussis D. The role of anti-eosinophilic therapies in eosinophilic granulomatosis with polyangiitis: a systematic review. Rheumatol Int. 2023;43:1245–52. [DOI] [PubMed] [PMC]

115.

Yilmaz A, Bauersachs J, Bengel F, Büchel R, Kindermann I, Klingel K, et al. Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK). Clin Res Cardiol. 2021;110:479–506. [DOI] [PubMed] [PMC]

116.

Toscano Peña A, Ali Munive A, Arevalo Y. Successful Treatment With Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A Case Report. Cureus. 2023;15:e38797. [DOI] [PubMed] [PMC]

117.

Sonneveld P. Management of multiple myeloma in the relapsed/refractory patient. Hematology Am Soc Hematol Educ Program. 2017;2017:508–17. [DOI] [PubMed] [PMC]

118.

Ahmed A, Killeen RB. Relapsed and Refractory Multiple Myeloma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. [PubMed]