Eosinophilic Granulomatosis with Polyangiitis (EGPA)

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

Diagnosis of EGPA Is a Complex Clinical Challenge

Patients can experience a variety of manifestations that impact multiple organ systems simultaneously.^1-3

Proportion of EGPA patients with clinical signs/symptoms^1-4

A silhouette with arrows pointing to different parts of the body where EGPA signs and symptoms are prevalent. Neuropathy occurs in 27-51% of patients, ENT involvement occurs in approximately 60-80% of patients, pulmonary signs (most commonly eosinophilic asthma) occurs in over 90% of patients, cutaneous symptoms (most commonly purpura) occur in 20-30% of patients, cardiac symptoms (most commonly cardiomyopathy) occurs in 30-45% of patients, renal involvement (most commonly glomerulonephritis) occurs in 5-12% of patients, and GI involvement (most commonly abdominal pain, diarrhea, and GI bleeding) occurs in approximately 20% of patients.

Most Common EGPA Manifestations

Eosinophilic Asthma

In most cases of EGPA, asthma is the first disease manifestation with >90% of patients having eosinophilic asthma. Asthma precedes the onset of systemic disease by a mean of 12 years.^2-4

Eosinophilic asthma in EGPA is classically severe, adult onset, and OCS dependent. At the time of diagnosis, the majority of patients with EGPA have severe asthma.^3,5

ENT Involvement

About 60 to 80% of patients with EGPA experience ENT manifestations. These manifestations typically emerge during the early phases of the disease.^4,6,7

Delayed and Missed Diagnoses

There are no commonly accepted diagnostic criteria for EGPA, and there is a lack of definitive biomarkers for EGPA.^2,8,9 These may factor into the frequent delayed or missed diagnoses as mean time from symptom onset to diagnosis is 12 years.^2,3

Common Differential Diagnoses Should Be Ruled Out

Symptomatic overlap with other diseases, including other forms of AAV, HES, and infection, can suggest that differential diagnoses need to be considered.^2,10,11

EGPA is distinct from MPA and GPA in that it is characterized by eosinophilia, asthma, and nasal polyps.^2,12

The distinction between EGPA and HES is challenging as both conditions are characterized by eosinophilia and widespread organ involvement.^2,10

Patients with HES usually do not have asthma or vasculitic complications, and they are ANCA negative.¹⁰

Helminthic infections should be ruled out, particularly in patients with GI involvement.

Eosinophilia and respiratory symptoms are major features of allergic bronchopulmonary aspergillosis and eosinophilic pneumonia. ²

Assessment Tools of EGPA

Organ Damage Is Frequent in Patients with EGPA

Organ damage accumulates over time in patients with EGPA.¹

Age, duration of OCS use, and prior relapse are associated with worse organ damage in patients with EGPA, as evidenced by higher VDI scores.¹

Organ damage substantially contributes to disease burden and mortality in patients with EGPA, as reflected by higher FFS.^1,16
- For example, patients with EGPA and cardiac insufficiency have a significantly increased risk of mortality (HR, 2.8; 95% CI, 1.2-5.9; p=0.02).

The most commonly reported manifestations of organ damage in patients with EGPA¹^,a

A horizontal bar chart showing the commonly reported manifestations of patients with EGPA. 67.5% of patients reported chronic asthma, 49.6% reported peripheral neuropathy, 31.3% reported chronic sinusitis/radiological damage, 27.2% reported nasal blockade/chronic discharge/crusting, 15.3% reported osteoporosis/vertebral collapse, 14.2% reported cataract, 13.4% reported chronic breathlessness, 11.6% reported hearing loss, and 11.2% reported cardiomyopathy.

^aVDI items present after at least 1 year of follow-up after diagnosis in a longitudinal cohort study (n=268).

Abbreviations

AAV = antineutrophil cytoplasmic antibody-associated vasculitis

;

ANCA = antineutrophil cytoplasmic antibody

;

BVAS = Birmingham Vasculitis Activity Score

;

CI = confidence interval

;

CNS = central nervous system

;

EGPA = eosinophilic granulomatosis with polyangiitis

;

ENT = ear, nose, and throat

;

FFS = Five Factor Score

;

GI = gastrointestinal

;

GPA = granulomatosis with polyangiitis

;

HES = hypereosinophilic syndrome

;

HR = hazard ratio

;

MPA = microscopic polyangiitis

; manually typed rather than reuse from abbreviation repository as this is a workaround provided by IT specific to how it is displayed in AEM;

OCS = oral corticosteroid(s)

;

QoL = quality of life

;

VDI = Vasculitis Damage Index

References

1. Doubelt I, Cuthbertson D, Carette S, et al. Clinical manifestations and long-term outcomes of eosinophilic granulomatosis with polyangiitis in North America. ACR Open Rheumatol. 2021;3(6):404-412. doi:10.1002/acr2.11263

2. Trivioli G, Terrier B, Vaglio A. Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. Rheumatology (Oxford). 2020;59(suppl 3):iii84-iii94. doi:10.1093/rheumatology/kez570

3. 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

4. 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

5. Khoury P, Grayson PC, Klion AD. Eosinophils in vasculitis: characteristics and roles in pathogenesis. Nat Rev Rheumatol. 2014;10(8):474-483. doi:10.1038/nrrheum.2014.98

6. Baldwin C, Wolter NE, Pagnoux C. Ear, nose, and throat involvement in eosinophilic granulomatosis with polyangiitis. Adv Cell Mol Otolaryngol. 2015;3(1):27181. doi:10.3402/acmo.v3.27181

7. Srouji I, Lund V, Andrews P, et al. Rhinologic symptoms and quality-of-life in patients with Churg-Strauss syndrome vasculitis. Am J Rhinol. 2008;22(4):406-409. doi:10.2500/ajr.2008.22.3204

8. 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. 2022;81(3):309-314. doi:10.1136/annrheumdis-2021-221794

9. 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

10. Gioffredi A, Maritati F, Oliva E, et al. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. doi:10.3389/fimmu.2014.00549

11. 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

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

13. Mukhtyar C, Lee R, Brown D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68(12):1827-1832. doi:10.1136/ard.2008.101279

14. Flossmann O, Bacon P, de Groot K, et al. Development of comprehensive disease assessment in systemic vasculitis. Ann Rheum Dis. 2007;66(3):283-292. doi:10.1136/ard.2005.051078

15. Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40(2):371-380. doi:10.1002/art.1780400222

16. 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). 2011;90(1):19-27. doi:10.1097/MD.0b013e318205a4c6

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

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References

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