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# Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

A rare vasculitis of small vessels in people with asthma and high eosinophils that can damage nerves, heart, lungs, skin and kidneys.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Nasal cavity](https://durgical.com/en/anatomy/nasal-cavity/)
- [Common fibular nerve](https://durgical.com/en/anatomy/common-fibular-nerve/)
- [Maxillary sinus](https://durgical.com/en/anatomy/maxillary-sinus/)
- [Main bronchi](https://durgical.com/en/anatomy/right-main-bronchus/)
- [Pericardium](https://durgical.com/en/anatomy/pericardium/)
- [Kidneys](https://durgical.com/en/anatomy/kidney/)
- [Sural nerves](https://durgical.com/en/anatomy/sural-nerve/)
- [Skin](https://durgical.com/en/anatomy/skin/)

## Why it happens

- [Granulomatous inflammation: caseating vs non-caseating, the causes to know](https://durgical.com/en/general-pathology/granulomatous-inflammation-caseating-vs-non-caseating-the-causes-to-know/)
- [White blood cells: types and functions](https://durgical.com/en/physiology/white-blood-cells-types-and-functions/)
- [Blood vessels: artery, vein and capillary layers](https://durgical.com/en/histology/blood-vessels-artery-vein-and-capillary-layers/)

## What it is

An ANCA-associated, necrotising small-vessel vasculitis with eosinophil-rich granulomatous inflammation. It typically develops in an adult with late-onset asthma and chronic rhinosinusitis with nasal polyps, followed by marked blood eosinophilia and then vasculitis affecting nerves, skin, heart, lungs, gut and kidneys.

## Who gets it

The rarest of the ANCA-associated vasculitides, with a few cases per 100,000 people. It usually appears between 40 and 60 years and affects men and women about equally. Latin American data are scarce; in Colombia it is managed in referral rheumatology centres.

## Causes

- Cause unknown; an autoimmune process with eosinophil and Th2 activation
- About 30–40% are positive for ANCA, mostly MPO-ANCA (p-ANCA)
- Genetic factors (HLA-DRB4) and environmental or allergic exposures are suspected
- Onset has occasionally followed reduction of corticosteroids in asthma or exposure to certain drugs

## Risk factors

- Late-onset or difficult-to-control asthma
- Chronic rhinosinusitis with nasal polyps and allergic rhinitis
- Middle age
- History of atopy

## Pathophysiology

Classically evolves in three overlapping phases: a prodromal allergic phase (asthma, rhinosinusitis), an eosinophilic phase (blood and tissue eosinophilia, pulmonary infiltrates, gastroenteritis) and a vasculitic phase. Eosinophils release toxic granule proteins that damage tissue, especially the heart; ANCA-positive patients more often have vasculitic features such as glomerulonephritis and neuropathy, whereas ANCA-negative patients more often have heart and lung disease.

## Classification

- Prodromal (allergic) phase: asthma, allergic rhinitis, nasal polyps
- Eosinophilic phase: blood eosinophilia, pulmonary infiltrates, eosinophilic gastroenteritis
- Vasculitic phase: mononeuritis multiplex, purpura, glomerulonephritis
- ANCA-positive versus ANCA-negative phenotypes

## Symptoms and signs

- Asthma that worsens or becomes harder to control in adulthood
- Nasal obstruction, polyps and chronic sinusitis
- Foot or wrist drop, numbness and pain from mononeuritis multiplex
- Palpable purpura and skin nodules
- Fever, weight loss, fatigue and muscle or joint pain
- Chest pain, breathlessness or palpitations from heart involvement; abdominal pain

## Red flags

- Signs of heart failure, chest pain or arrhythmia (eosinophilic myocarditis, the main cause of death)
- Haemoptysis or rapidly increasing breathlessness
- Rising creatinine, haematuria or proteinuria
- New foot drop or rapidly spreading neuropathy
- Severe abdominal pain or gastrointestinal bleeding (mesenteric vasculitis)

## Diagnosis

**Clinical**
Suspected in an adult with asthma, eosinophilia above 10% (or above 1,500 cells/µL) and features of vasculitis such as mononeuritis multiplex. Classification uses the 2022 ACR/EULAR criteria, which weight obstructive airway disease, nasal polyps, mononeuritis multiplex and eosinophilia.

**Tests**
Complete blood count with marked eosinophilia; raised IgE, ESR and CRP; ANCA (MPO by ELISA) positive in a minority; urinalysis and creatinine; troponin, ECG and NT-proBNP; stool tests and serology to exclude Strongyloides before steroids. Biopsy ([skin](https://durgical.com/en/anatomy/skin/), nerve, lung) shows eosinophil-rich necrotising vasculitis or granulomas; nerve conduction studies for neuropathy.

**Imaging**
[Chest](https://durgical.com/en/anatomy/region/chest/) radiograph or CT showing migrating patchy infiltrates; sinus CT; echocardiography and cardiac MRI to detect myocarditis.

## Differential diagnosis

- Granulomatosis with polyangiitis and microscopic polyangiitis
- Chronic eosinophilic pneumonia and hypereosinophilic syndrome
- Allergic bronchopulmonary aspergillosis
- Parasitic infection such as strongyloidiasis
- Drug reaction with eosinophilia
- Polyarteritis nodosa

## Goal of treatment

Induce remission quickly to protect the heart, nerves and kidneys, then maintain remission with the least corticosteroid possible while keeping asthma controlled.

## Treatment

**Acute**
Severe organ-threatening disease (heart, [kidney](https://durgical.com/en/anatomy/kidney/), nerve, gut): high-dose systemic corticosteroids, often intravenous pulses, with cyclophosphamide or rituximab for induction.

**Medical**
Non-severe disease: systemic corticosteroids, adding anti-IL-5 therapy (mepolizumab, benralizumab) to control eosinophils and asthma and spare steroids.

**Medical**
Maintenance with azathioprine, methotrexate or anti-IL-5 therapy; inhaled therapy for asthma; treatment of Strongyloides before immunosuppression in endemic areas such as Colombia.

**Follow-up**
Monitoring of eosinophil count, ESR, kidney function, heart status and neuropathy; nursing care for falls with foot drop, infection prevention and steroid side effects (glucose, blood pressure, bone).

## Complications

- Eosinophilic myocarditis, cardiomyopathy and heart failure
- Permanent peripheral neuropathy
- Glomerulonephritis and kidney failure
- Intestinal ischaemia or perforation
- Steroid dependence because of persistent asthma

## Prognosis

With treatment most patients reach remission and five-year survival exceeds 90%, but relapses are common and many need long-term low-dose corticosteroids for asthma. Heart involvement is the main determinant of poor outcome.

## Test yourself

Think of the answer, then tap the question to see it.

## What triad suggests eosinophilic granulomatosis with polyangiitis?

Adult-onset asthma, marked peripheral eosinophilia and systemic vasculitis (for example mononeuritis multiplex).

## Which ANCA pattern is most associated with EGPA, and how often is it positive?

p-ANCA (anti-MPO), positive in only about a third to 40% of patients.

## What is the leading cause of death in EGPA?

Cardiac involvement (eosinophilic myocarditis or cardiomyopathy).

## Which parasitic infection should be excluded or treated before high-dose steroids in a patient with eosinophilia from Colombia?

Strongyloidiasis, to avoid hyperinfection syndrome.

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## Related conditions

- [Asthma](https://durgical.com/en/conditions/asthma/)
- [Chronic rhinosinusitis and nasal polyps](https://durgical.com/en/conditions/chronic-rhinosinusitis-and-nasal-polyps/)
- [Eosinophilic pneumonias (including Löffler syndrome)](https://durgical.com/en/conditions/eosinophilic-pneumonias/)
- [Allergic bronchopulmonary aspergillosis (ABPA)](https://durgical.com/en/conditions/allergic-bronchopulmonary-aspergillosis/)
- [Strongyloidiasis](https://durgical.com/en/conditions/strongyloidiasis/)
- [Granulomatosis with polyangiitis](https://durgical.com/en/conditions/granulomatosis-with-polyangiitis/)
- [Microscopic polyangiitis](https://durgical.com/en/conditions/microscopic-polyangiitis/)
- [Peripheral neuropathy (diabetic and other)](https://durgical.com/en/conditions/peripheral-neuropathy/)
- [Myocarditis](https://durgical.com/en/conditions/myocarditis/)
- [Dilated cardiomyopathy](https://durgical.com/en/conditions/dilated-cardiomyopathy/)
- [Rapidly progressive glomerulonephritis (anti-GBM, ANCA)](https://durgical.com/en/conditions/rapidly-progressive-glomerulonephritis/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)](https://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/) (Johns Hopkins Vasculitis Center, 2026); [Vasculitis](https://medlineplus.gov/vasculitis.html) (MedlinePlus (U.S. National Library of Medicine), 2026).

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Source: https://durgical.com/en/conditions/eosinophilic-granulomatosis-with-polyangiitis/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/eosinophilic-granulomatosis-with-polyangiitis/