Eosinophilic Granulomatosis with Polyangiitis (EGPA)

AstraZeneca Medical's ambition is to address unmet needs for people living with inflammatory diseases driven by eosinophilic inflammation.

Managing EGPA

There is an unmet need for earlier, optimized management of EGPA.^1-6

EGPA treatment considerations include controlling inflammation, achieving long-term remission^a, and preventing disease relapse, while minimizing dose- and drug-related toxicity.

Eosinophilic Inflammation Is a Defining Feature of EGPA^7,8

GOAL: Reduce eosinophilic inflammation

EGPA Is a Chronic Condition^9,10

GOAL: Achieve long-term remission, using well-tolerated treatments, with minimal drug toxicity^a

Disease Relapse Is Common^11,12

GOAL: Prevent disease relapse, particularly during steroid tapering

^aNote that definitions for remission vary between established guidelines and clinical trials.

Early Collaboration Can Help Optimize Disease Management

A wheel of different healthcare specialties representing the multidisciplinary coordinated care team that can be involved in the management of patients with EGPA. The team includes cardiology, dermatology, ENT specialists, gastroenterology, general medicine, neurology, nephrology, respiratory medicine, and rheumatology.

EGPA is most frequently diagnosed by rheumatologists and pulmonologists¹³; however, other disciplines may also be involved in diagnosis.^5,14,15 Early collaboration can help optimize disease management to enhance outcomes for patients.⁴

The 2022 EULAR guidelines deem multidisciplinary management by specialist centers with expertise in vasculitis a fundamental principle for the management of EGPA.⁴

Patients with EGPA experience substantial disease burden^1-3,9,16

Cyclical figure depicting the disease burden patients with EGPA experience. At the top, there is an icon of a clipboard with danger symbol indicating risk for treatment-related adverse events with long-term use of OCS and immunosuppressants. To the right is an icon of a hospital indicating that patients can experience frequent medical visits, hospitalization and ED visits. Below, is an icon of a body silhouette pointing out different areas of the body indicating that comorbidities are frequent and patients can have a variety of systemic manifestations of EGPA. Next is an icon of a speed dial with 3 emoji faces (happy, neutral, and sad) indicating reduced HRQoL across all eight dimensions of SF-36. The last icon is a medical cross with arrows in a cycle indicating that up to 81% of patients with EGPA experience disease relapse.

Treatment Strategies

OCS and Non-Specific Immunosuppressants

High-dose OCS therapy is a cornerstone of treatment for EGPA, while non-specific immunosuppressants are used to induce and/or maintain remission.

Most patients are prescribed OCS and receive them long term.^3,4,17

Adverse events that may be associated with long-term use of OCS^18-20

$Adverse events include osteoporosis and bone fracture (shattered bone icon), diabetes (pancreas icon), hypertension (heart icon), cataracts (eye icon), infections (virus icon).$

Adverse events typically associated with long-term use of non-specific immunosuppressants^21,22

Adverse events include infection (virus icon), bone marrow suppression and cytopenia (bone marrow icon), cardiotoxicity (heart icon), malignancy (malignancy icon), liver toxicity (liver icon), and myelosuppression (bone zooming into cells).

OCS and immunosuppressants can be associated with a risk of side effects to patients, and the disease frequently remains uncontrolled. To prevent or reduce toxicity, it is recommended to taper OCS dose once remission has been achieved; however, disease relapse is common during tapering.^3,4,11

Advances in Therapy

Advances in the understanding of EGPA pathophysiology have led to additional treatment approaches. Biologics targeting eosinophils are an emerging treatment strategy based on advances in the understanding of the pathophysiology of EGPA.¹⁴

Treatment Algorithms for EGPA

Abbreviations

ACR = American College of Rheumatology

;

CNS = central nervous system

;

ED = emergency department

;

EGPA = eosinophilic granulomatosis with polyangiitis

;

ENT = ear, nose, and throat

;

EULAR = European Alliance of Associations for Rheumatology

;

GI = gastrointestinal

;

HRQoL = health-related quality of life

;

OCS = oral corticosteroid(s)

;

SF-36 = 36-item short-form health survey

References

1. Jackson DJ, Akuthota P, Roufosse F. Eosinophils and eosinophilic immune dysfunction in health and disease. Eur Respir Rev. 2022;31(163):210150. doi:10.1183/16000617.0150-2021

2. Jakes RW, Kwon N, Nordstrom B, et al. Burden of illness associated with eosinophilic granulomatosis with polyangiitis: a systematic literature review and meta-analysis. Clin Rheumatol. 2021;40(12):4829-4836. doi:10.1007/s10067-021-05783-8

3. 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. 2021;73(8):1366-1383. doi:10.1002/art.41773

4. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83(1):30-47. doi:10.1136/ard-2022-223764

5. Bloom JL, Langford CA, Wechsler ME. Therapeutic advances in eosinophilic granulomatosis with polyangiitis. Rheum Dis Clin North Am. 2023;49(3):563-584. doi:10.1016/j.rdc.2023.03.006

6. Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17(1):60-64. doi:10.7861/clinmedicine.17-1-60

7. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi:10.1002/art.37715

8. Fagni F, Bello F, Emmi G. Eosinophilic granulomatosis with polyangiitis: dissecting the pathophysiology. Front Med (Lausanne). 2021;8:627776. doi:10.3389/fmed.2021.627776

9. Sokołowska B, Szczeklik W, Piłat O, et al. The impact of current health-related quality of life on future health outlook in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Clin Rheumatol. 2013;32(6):779-785. doi:10.1007/s10067-013-2169-7

10. Benarous L, Terrier B, Laborde-Casterot H, et al. Employment, work disability and quality of life in patients with ANCA-associated vasculitides. The EXPOVAS study. Clin Exp Rheumatol. 2017;35(1)(suppl 103):S40-S46.

11. Ribi C, Cohen P, Pagnoux C, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58(2):586-594. doi:10.1002/art.23198

12. Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-1594. doi:10.1136/annrheumdis-2016-209133

13. Chen LF, Qianhua LI, Zheng DH, et al. Comparison of clinical characteristics of eosinophilic granulomatosis with polyangiitis between rheumatology and respiratory medicine: a single center, retrospective study [abstract]. Ann Rheum Dis. 2019;78(suppl 2):1751-1752. Abs AB0579. doi:10.1136/annrheumdis-2019-eular.5369

14. Emmi G, Bettiol A, Gelain E, et al. Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023;19(6):378-393. doi:10.1038/s41584-023-00958-w

15. Vasculitis Foundation. Pediatric vasculitis. Who gets EGPA? Vasculitis Foundation website. https://www.vasculitisfoundation.org/pediatrics-vasculitis/pediatric-types/pediatric-eosinophilic-granulomatosis-with-polyangiitis/

16. Cottin V, Bel E, Bottero P, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J. 2016;48(5):1429-1441. doi:10.1183/13993003.00097-2016

17. Jakes R, Kwon N, Huynh L, et al. Characterisation and burden of eosinophilic granulomatosis with polyangiitis: a European retrospective study [abstract]. Eur Respir J. 2022;60(suppl 66):2254. doi:10.1183/13993003.congress-2022.2254

18. Rice JB, White AG, Scarpati LM, et al. Long-term systemic corticosteroid exposure: a systematic literature review. Clin Ther. 2017;39(11):2216-2229. doi:10.1016/j.clinthera.2017.09.011

19. Baldini C, Talarico R, Della Rossa A, et al. Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin North Am. 2010;36(3):527-543. doi:10.1016/j.rdc.2010.05.003

20. Scherbacher PJ, Hellmich B, Feng Y-S, et al. Prospective study of complications and sequelae of glucocorticoid therapy in ANCA-associated vasculitis. RMD Open. 2024;10(1):e003956. doi:10.1136/rmdopen-2023-003956

21. Alam V, Nanzer AM. Eosinophilic granulomatosis with polyangiitis: case report and literature review. Breathe (Sheff). 2022;18(4):220170. doi:10.1183/20734735.0170-2022

22. Hsu DC, Katelaris CH. Long-term management of patients taking immunosuppressive drugs. Aust Prescr. 2009;32(3):68-71. doi:10.18773/austprescr.2009.035

US-88106, US-91275 Last Updated 7/24

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Abbreviations

References

Welcome to AstraZeneca Medical Information

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