Idiopathic pulmonary fibrosis

IPF

idiopathic pulmonary fibrosis: Idiopathic pulmonary fibrosis (IPF) is a nonneoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known cause.

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Endpoint definition

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 J84.1
  • Cause of death: ICD-10 J84.1

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

2495

4. Check minimum number of events

None

2495

5. Include endpoints

None

2495

6. Filter based on genotype QC (FinnGen only)

2401

Control definitions (FinnGen only)

Control exclude ILD_ENDPOINTS

Extra metadata

First used in FinnGen datafreeze DF5
Parent code in ICD-10 J84
Name in latin Morbus pulmonum interstitialis cum fibrosi

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 18549 7438 10834
Only index persons 13181 5367 7814
Unadjusted period prevalence (%)
Whole population 0.27 0.21 0.30
Only index persons 0.26 0.20 0.30
Median age at first event (years)
Whole population 74.28 75.14 73.71
Only index persons 74.01 74.46 73.69

-FinnGen-

Key figures

All Female Male
Number of individuals 2401 808 1593
Unadjusted period prevalence (%) 0.64 0.32 0.80
Median age at first event (years) 71.61 69.42 72.73

-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

Association

Association between endpoint IPF and mortality.

Females

Parameter HR [95% CI] p-value
IPF 3.691 [3.29, 4.14] < 0.001
Birth year 0.992 [0.98, 1.0] 0.045

During the follow-up period (1.1.1998 — 31.12.2019), 3391 out of 5474 females with IPF died.

Males

Parameter HR [95% CI] p-value
IPF 3.607 [3.15, 4.13] < 0.001
Birth year 0.985 [0.98, 0.99] 0.001

During the follow-up period (1.1.1998 — 31.12.2019), 5042 out of 7688 males with IPF died.

Mortality risk

Mortality risk for people of age

years, who have IPF.

N-year risk Females Males
1 0.269% 0.673%
5 1.736% 3.833%
10 4.577% 9.627%
15 8.667% 17.651%
20 15.51% 28.109%

Relationships between endpoints

Index endpoint: IPF – Idiopathic pulmonary fibrosis

GWS hits: 10

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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