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Wegener granulomatosis

DOID EFO Endpoint Browser PheWeb

M13_WEGENER

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

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Endpoint definition

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Endpoint definition steps

FinnGen

Phenotype data

473681

1. Apply sex-specific rule

None

473681

2. Check conditions

None

473681

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

Registry filters:

  • Hospital discharge: ICD-10 — M31.3
  • Hospital discharge: ICD-9 — 4464
  • Hospital discharge: ICD-8 — 4462
  • Cause of death: ICD-10 — M31.3
  • Cause of death: ICD-9 — 4464
  • Cause of death: ICD-8 — 4462

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

503

4. Check minimum number of events

None

503

5. Include endpoints

None

503

6. Filter based on genotype QC (FinnGen only)

486

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 M31
Name in latin Granulomatosis Wegener

Case counts by codes

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FinnGen case counts by registry codes:

generic upset plot

Upset plot

Full data table

Summary Statistics

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-FinRegistry-

Key figures

All Female Male
Number of individuals
Whole population 2962 1497 1441
Only index persons 2449 1266 1183
Unadjusted period prevalence (%)
Whole population 0.04 0.04 0.04
Only index persons 0.05 0.05 0.05
Median age at first event (years)
Whole population 60.38 61.29 59.32
Only index persons 59.11 60.06 58.09

-FinnGen-

Key figures

All Female Male
Number of individuals 486 249 237
Unadjusted period prevalence (%) 0.11 0.10 0.12
Median age at first event (years) 55.29 54.44 56.19

-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

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Association

Association between endpoint M13_WEGENER and mortality.

Females

Parameter HR [95% CI] p-value
M13_WEGENER 2.443 [2.07, 2.88] < 0.001
Birth year 0.99 [0.98, 1.0] 0.026

During the follow-up period (1.1.1998 — 31.12.2019), 351 out of 1165 females with M13_WEGENER died.

Males

Parameter HR [95% CI] p-value
M13_WEGENER 2.398 [2.01, 2.86] < 0.001
Birth year 0.983 [0.97, 0.99] < 0.001

During the follow-up period (1.1.1998 — 31.12.2019), 368 out of 1088 males with M13_WEGENER died.

Mortality risk

Mortality risk for people of age

years, who have M13_WEGENER.

N-year risk Females Males
1 0.245% 0.406%
5 1.471% 2.311%
10 3.471% 6.235%
15 6.26% 11.394%
20 10.164% 18.006%

Relationships between endpoints

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Index endpoint: M13_WEGENER – Wegener granulomatosis

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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