Polyarteritis with lung involvement [Churg-Strauss]/EGPA

M13_CHURGSTRAUSS

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.

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Endpoint definition

Endpoint definition steps

FinnGen

Phenotype data

429209

1. Apply sex-specific rule

None

429209

2. Check conditions

None

429209

3. Check pre-conditions, main-only, mode, registry filters

Registry filters:

  • Hospital discharge: ICD-10 M30.1
  • Hospital discharge: ICD-9 4460B
  • Cause of death: ICD-10 M30.1
  • Cause of death: ICD-9 4460B

2 out of 7 registries used, show all original rules.

111

4. Check minimum number of events

None

111

5. Include endpoints

None

111

6. Filter based on genotype QC (FinnGen only)

107

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

Case counts by codes

FinnGen case counts by registry codes:

generic upset plot

Upset plot

Full data table

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 107 69 38
Unadjusted period prevalence (%) 0.03 0.03 0.02
Median age at first event (years) 53.35 52.75 54.44

-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

Mortality – FinRegistry

Relationships between endpoints

Index endpoint: M13_CHURGSTRAUSS – Polyarteritis with lung involvement [Churg-Strauss]/EGPA

GWS hits: -

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