Wegener granulomatosis

Description

Granulomatosis with Polyangiitis: A small-vessel necrotizing vasculitis characterised by the association of inflammation of the vessel wall and peri- and extravascular granulomatosis.

Data source
FinnGen phenocode M13_WEGENER
Hospital Discharge registry ICD-10: M313, ICD-8: 4462
Cause of Death registry ICD-10: M313, ICD-8: 4462
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 12132
GWAS catalog 0005297
MESH D014890
SNOMED CT 195353004

Key figures

Sex All Female Male
Number of individuals 121 63 58
Unadjusted prevalence (%) 0.09 0.08 0.1
Mean age at first event (years) 53.75 52.53 55.08
Median number of events / individual 8 3 20

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 71.07 63.49 79.31
Case fatality at 5-years (%) 4.96 4.76 5.17

Associations