EGPA (Eosinophilic Granulomatosis with Polyangiitis)

Exam Cram

• EGPA (Eosinophilic Granulomatosis with Polyangiitis): small vessel vasculitis

• Presentation- asthma + eosinophilia + haematoproteinuria

• Investigations: pANCA, ANA +ve, raised CRP

• Management: steroids

Outline

Background

• Prevalence: 1 case per million

• Age of presentation: 50yrs

Presentation

• General malaise, weight loss, fevers, nasal discharge

• Background: asthma

Examination

• Peripheral neuropathies (mononeuritis)

• Purpuric rash

• Chest auscultation: scattered wheeze

Investigations

• pANCA: +ve (sensitivity 40%, specificity high)

• Urinanalysis: blood & protein

• Eosinophils: raised

Differentials

• Asthma exacerbation

• ABPA

• Ig4 Deficiency

Diagnosis

• Clinical but helped by biopsy, of e.g. skin

• See Med reg notes

Management

1. High dose steroids

2. Cyclophosphamide

Med Reg Notes

Background

• Similar male: female prevalence

• Wegner's Granulomatous was renamed to GPA due to his links to the Nazi party. However Churg Strauss was a Jewish Pole who emigrated to the USA. The disease is renamed to ensure consistency across the vasculitidies. 

Histology

EGPA is defined histologically be having

1. Tissue rich Eosinophilia with granulomas

2. Necrotising vasculitis

Diagnostic Criteria

ACR-EULAR: American and European Rheumatology Society Diagnostic criteria 2022

Points > 5: sensitivity 85%, specificity 99%

1. Eosinophils > 1 x10^9: +5

2. Obstructive airway disease: +3

3. Nasal polyps +3

4. Extravascular eosinophilic inflammation +2

5. Mononeuritis multiplex +1

6. ANCA positive -3

7. Haematuria -1

Management [1]

First presentation

• Well: steroids

• Unwell: steroids + cyclophosphamide

Relapsing

• Well: steroids + mepolizumab

• Unwell: steroids + cyclophosphamide + rituxumab

Different names

• P-ANCA: MPO

• C-ANCA: PR3

References and Resources

1. https://www.nature.com/articles/s41584-023-00958-w

2. Chakraborty RK, Aeddula NR. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) [Updated 2024 Sep 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537099/

Written in 2024