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Start free with EleplanGranulomatosis with polyangiitis
ORPHA:900Disease
Also called GPA · Wegener granulomatosis
What it is
A rare anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis characterized by necrotizing inflammation of small and medium vessels (capillaries, venules and arterioles), resulting in tissue ischemia.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adolescent, Adult, Childhood, 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%
17Common30–79%
28- Abdominal pain
- Abnormality of the hypothalamus-pituitary axis
- Anti-myeloperoxidase antibody positivity
- Anti-neutrophil elastase antibody positivity
- Anti-proteinase 3 antibody positivity
- Arthritis
- Chest pain
- Chronic pulmonary obstruction
- Cough
- Cytoplasmic antineutrophil antibody positivity
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Glomerulonephritis
- Hemoptysis
- Increased inflammatory response
- Inflammatory abnormality of the eye
- Nasolacrimal duct obstruction
- Nausea and vomiting
- Papule
- Periorbital edema
- Peripheral neuropathy
- Polyarticular arthritis
- Proteinuria
- Pulmonary fibrosis
- Recurrent intrapulmonary hemorrhage
- Respiratory insufficiency
- Scleritis
- Skin rash
Sometimes5–29%
35- Angina pectoris
- Arrhythmia
- Chronic otitis media
- Cranial nerve paralysis
- Diabetes insipidus
- Diffuse alveolar hemorrhage
- Gangrene
- Gastrointestinal hemorrhage
and 27 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.
Genes
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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