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.
Polyarteritis with lung involvement [Churg-Strauss]/EGPA
DOID EFO Endpoint Browser PheWeb
M13_CHURGSTRAUSS
Endpoint definition
↥
Endpoint definition steps |
FinnGen |
|---|---|
Phenotype data |
473681 |
1. Apply sex-specific ruleNone |
473681 |
2. Check conditionsNone |
473681 |
3. Check pre-conditions, main-only, mode, registry filtersRegistry filters:
2 out of 7 registries used, show all original rules. |
124 |
4. Check minimum number of eventsNone |
124 |
5. Include endpointsNone |
124 |
6. Filter based on genotype QC (FinnGen only) |
118 |
Control definitions (FinnGen only)
Control exclude
M13_SYSTCONNECT
Extra metadata
Level in the ICD hierarchy
4
First used in FinnGen datafreeze
DF2
Parent code in ICD-10
M30
Name in latin
Polyarteritis nodosa cum manifestationibus pulmonalibus
Similar endpoints
↥List of similar endpoints to
Polyarteritis with lung involvement [Churg-Strauss]/EGPA
based on the number of shared cases.
Similar with more cases:
- Polyarteritis nodosa and related conditions
- Systemic connective tissue disorders
- Autoimmune diseases related-to ILD
- Autoimmune diseases
- Used for control exclusion in common control thyroid runs
Similar with less cases:
None
Case counts by codes
↥
Summary Statistics
↥-FinRegistry-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | |||
| Whole population | 674 | 428 | 243 |
| Only index persons | 564 | 368 | 196 |
| Unadjusted period prevalence (%) | |||
| Whole population | 0.01 | 0.01 | 0.01 |
| Only index persons | 0.01 | 0.01 | 0.01 |
| Median age at first event (years) | |||
| Whole population | 59.17 | 58.51 | 60.28 |
| Only index persons | 57.40 | 56.70 | 58.70 |
-FinnGen-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | 118 | 76 | 42 |
| Unadjusted period prevalence (%) | 0.03 | 0.03 | 0.02 |
| Median age at first event (years) | 53.73 | 52.68 | 55.63 |
-FinRegistry-
Age distribution of first events
-FinnGen-
Age distribution of first events
-FinRegistry-
Year distribution of first events
-FinnGen-
Year distribution of first events
-FinRegistry-
Cumulative Incidence Function
-FinnGen-
Cumulative Incidence Function
No data
Mortality – FinRegistry
↥Association
Association between endpoint M13_CHURGSTRAUSS and mortality.
Females
| Parameter | HR [95% CI] | p-value |
|---|---|---|
| M13_CHURGSTRAUSS | 1.776 [1.31, 2.4] | < 0.001 |
| Birth year | 0.997 [0.99, 1.01] | 0.509 |
During the follow-up period (1.1.1998 — 31.12.2019), 95 out of 338 females with M13_CHURGSTRAUSS died.
Males
| Parameter | HR [95% CI] | p-value |
|---|---|---|
| M13_CHURGSTRAUSS | 1.771 [1.23, 2.56] | 0.002 |
| Birth year | 0.988 [0.98, 1.0] | 0.009 |
During the follow-up period (1.1.1998 — 31.12.2019), 54 out of 192 males with M13_CHURGSTRAUSS died.
Mortality risk
Mortality risk for people of age
years, who have M13_CHURGSTRAUSS.| N-year risk | Females | Males |
|---|---|---|
| 1 | 0.195% | 0.339% |
| 5 | 1.128% | 2.094% |
| 10 | 2.675% | 5.255% |
| 15 | 5.195% | 9.979% |
| 20 | 9.422% | 16.502% |
Relationships between endpoints
↥Index endpoint: M13_CHURGSTRAUSS – Polyarteritis with lung involvement [Churg-Strauss]/EGPA
GWS hits: -
Endpoint
Case Overlap
Survival Analysis
Genetic Correlations
Genetic Signals
N (Jaccard index)
HR [CI]
Extremity
rg [CI]
Extremity
Hits
Coloc Hits
FinRegistry
FinnGen
FinRegistry
FinnGen
FinnGen
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