granulomatosis with polyangiitis
MONDO:0012105Mondo
Findings
No curated finding names granulomatosis with polyangiitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A small-vessel necrotizing vasculitis characterized by the association of inflammation of the vessel wall and peri- and extravascular granulomatosis.
Definition from the Mondo Disease Ontology (MONDO:0012105), read 2026-09-29. CC BY 4.0.
- Inheritance
- Polygenic inheritance
- Onset and course
- Middle age onset · Pediatric onset
HPO, annotations 2026-09-02
Features
95 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Cytoplasmic antineutrophil antibody positivityHPOHP:0032230
- 283 of 331 reported patients
- Frequent (30% to 79% of cases)
- Abnormal oral cavity morphologyHPOHP:0000163
- Very frequent (80% to 99% of cases)
- Abnormality of the noseHPOHP:0000366
- Very frequent (80% to 99% of cases)
- ArthralgiaHPOHP:0002829
- Very frequent (80% to 99% of cases)
- AutoimmunityHPO · MondoHP:0002960
- Very frequent (80% to 99% of cases)
- Cerebral ischemiaHPOHP:0002637
- Very frequent (80% to 99% of cases)
Show the remaining 83
- Otitis mediaHPOHP:0000388
- Very frequent (80% to 99% of cases)
- Pulmonary infiltratesHPOHP:0002113
- Very frequent (80% to 99% of cases)
- Recurrent respiratory infectionsHPOHP:0002205
- Very frequent (80% to 99% of cases)
- SinusitisHPOHP:0000246
- Very frequent (80% to 99% of cases)
- VasculitisHPO · MondoHP:0002633
- Very frequent (80% to 99% of cases)
- Weight lossHPOHP:0001824
- Very frequent (80% to 99% of cases)
Where it sits
Other names
7 names
Resolves to: granulomatosis with polyangiitis
- Also called
- GPAgranulomatosis - Wegener'snecrotizing respiratory granulomatosispauci-immune glomerulonephritis associated with granulomatosis with polyangiitisWegener granulomatosisWegener's granulomatosisWegener's syndrome