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Start free with EleplanEosinophilic granulomatosis with polyangiitis
ORPHA:183Disease
Also called Churg-Strauss syndrome · EGPA · Granulomatous allergic angiitis
What it is
A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations.
Key facts
- Prevalence
- 1-9 / 100 000
- Age of onset
- Adolescent, Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
12Common30–79%
24- Abdominal pain
- Abnormality of the pleura
- Abnormal pericardium morphology
- Airway obstruction
- Anti-myeloperoxidase antibody positivity
- Anti-neutrophil elastase antibody positivity
- Anti-proteinase 3 antibody positivity
- Arthralgia
- Cytoplasmic antineutrophil antibody positivity
- Dysphagia
- Eosinophilic pneumonia
- Fatigue
- Gait disturbance
- Hematuria
- Hypertension
- Hypertrophic cardiomyopathy
- Hypopigmented skin patches
- Increased circulating IgE concentration
- Multiple mononeuropathy
- Nausea and vomiting
- Rheumatoid factor positive
- Skin rash
- Tubulointerstitial nephritis
- Venous thrombosis
Sometimes5–29%
30- Acrocyanosis
- Arthritis
- Congestive heart failure
- Cough
- Cranial nerve paralysis
- Crescentic glomerulonephritis
- Cutaneous granuloma
- Cutis marmorata
and 22 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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