Polyarteritis with lung involvement [Churg-Strauss]/EGPA
Description
Churg-Strauss syndrome: Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia.
Data source
| FinnGen phenocode | M13_CHURGSTRAUSS |
| Hospital Discharge registry | ICD-10: M301, ICD-9: 4460B |
| Cause of Death registry | ICD-10: M301, ICD-9: 4460B |
| Level in the ICD-hierarchy | 4 |
| First defined in version | DF2 |
Key figures
| Sex | All | Female | Male |
| Number of individuals | 27 | 19 | 8 |
| Unadjusted prevalence (%) | 0.02 | 0.03 | 0.01 |
| Mean age at first event (years) | 49.13 | 49.76 | 47.63 |
| Median number of events / individual | 7 | 2 | 16 |
Clinical metrics
| Sex | All | Female | Male |
| Recurrence within 6 months (%) | 59.26 | 52.63 | 75.0 |
| Case fatality at 5-years (%) | 7.41 | 5.26 | 12.5 |