Metadata
| Name | Other interstitial pulmonary diseases with fibrosis (pulm_fibrosis) |
| Category | Respiratory |
| Sex | |
| Adult | Yes |
| UUID | deac8362-2059-4cd9-b43f-30533d5734cb |
| Definition | 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. |
Ontologies
| Disease Ontology (DOID) | DOID_0050156 |
| GWAS Catalogue | EFO_0000768 |
| MeSH | D054990 |
| SNOMED-CT | 700250006 |
| FinnGen | IPF |
Key Summary Statistics
Cases from any source
| Total | 3320 |
| Female (%) | 1292 (39%) |
| Male (%) | 2028 (61%) |
Year first recorded
Cases linked in all UK Biobank sources
Cross-source concordance
Vertical bars correspond to number of cases per source/intersection; horizontal bars to left correspond to total number of cases per source.
Incidence & prevalence
Baseline prevalence stratified by sex
| Female | 10.73 (8.52,12.93) |
| Male | 20.02 (16.74,23.3) |
Incidence stratified by sex
| Female | 2.39 (1.99,2.79) |
| Male | 4.09 (3.52,4.67) |
Incidence stratified by age
| Ages 40-44 | 0.5 (0.06,0.94) |
| Ages 45-49 | 0.82 (0.33,1.3) |
| Ages 50-54 | 1.27 (0.71,1.82) |
| Ages 55-59 | 1.56 (1.0,2.12) |
| Ages 60-64 | 4.43 (3.62,5.23) |
| Ages 65-69 | 7.51 (6.3,8.71) |
Period prevalence
Methods
Comparison with general population
Sex-standardised prevalence from CALIBER study (Kuan et al. 2019)
| Population | Ages 40-49 | Ages 50-59 | Ages 60-69 |
| UK Biobank | 7 (4,10) | 12 (10,15) | 44 (40,49) |
| CALIBER | 6 (5,7) | 16 (14,17) | 45 (43,47) |
Stratified by age and Townsend deprivation status
| Deprivation status | Ages 40-49 | Ages 50-59 | Ages 60-69 |
| Least deprived | 6(2,15) | 5(2,11) | 45(36,55) |
| Low | 4(1,12) | 11(6,18) | 40(31,50) |
| Medium | 7(2,17) | 9(5,17) | 44(35,54) |
| High | 6(2,14) | 12(6,19) | 43(33,54) |
| Most deprived | 9(4,17) | 24(16,34) | 50(40,63) |
Stratified by age and country
| Country | Ages 40-49 | Ages 50-59 | Ages 60-69 |
| England | 6(4,10) | 11(9,15) | 42(37,47) |
| Scotland | 6(1,19) | 14(7,24) | 51(38,67) |
| Wales | 10(2,29) | 17(7,33) | 61(44,82) |
Risk factors analysis
‘Healthy’ BMI, smoking status ‘never’, or no hypertension (absence of hypertension records) were considered as reference values.
Hazard Ratio (HR) values with P < 0.0002 were considered to show significant association with disease onset after correction for multiple-testing using the Bonferroni method
N=153513, Events=116
| Factor | Level | Hazard Ratio | P value |
| BMI | overweight | 1.85 (1.14, 2.99) | 0.0128 |
| BMI | obese | 1.9 (1.12, 3.22) | 0.0174 |
| BMI | underweight | 0 (0, Inf) | 0.993 |
| smoking | previous | 0.82 (0.53, 1.27) | 0.367 |
| smoking | current | 1.79 (1.09, 2.95) | 0.0215 |
| hypertension | yes | 1.55 (1.01, 2.37) | 0.0432 |
Definition
| UK Biobank field id | Description | Code | Value |
|---|---|---|---|
| 22175 | Recent medication for idiopathic pulmonary fibrosis | 1 | Yes |
| 22135 | Doctor diagnosed idiopathic pulmonary fibrosis | 1 | Yes |
| 20002 | Non-cancer illness code, self-reported | 1115 | interstitial lung disease |
| 20002 | Non-cancer illness code, self-reported | 1122 | fibrosing alveolitis/unspecified alveolitis |
| 20002 | Non-cancer illness code, self-reported | 1121 | pulmonary fibrosis |
| 41202 | Diagnoses - main ICD10 | J84.1 | Other interstitial pulmonary diseases with fibrosis |
| 41204 | Diagnoses - secondary ICD10 | J84.1 | Other interstitial pulmonary diseases with fibrosis |
| 40001 | Underlying (primary) cause of death: ICD10 | J84.1 | Other interstitial pulmonary diseases with fibrosis |
| 40002 | Contributory (secondary) causes of death: ICD10 | J84.1 | Other interstitial pulmonary diseases with fibrosis |
| 42040 | GP clinical event records | 23E5.00 | O/E - fibrosis of lung present |
| 42040 | GP clinical event records | 23E5.11 | O/E - fibrosis of lung |
| 42040 | GP clinical event records | H55..00 | Postinflammatory pulmonary fibrosis |
| 42040 | GP clinical event records | H55..11 | Cirrhosis of lung |
| 42040 | GP clinical event records | H563100 | Diffuse pulmonary fibrosis |
| 42040 | GP clinical event records | H563200 | Pulmonary fibrosis |
| 42040 | GP clinical event records | H563300 | Usual interstitial pneumonitis |
| 42040 | GP clinical event records | H563.00 | Idiopathic fibrosing alveolitis |
| 42040 | GP clinical event records | H563.11 | Hamman - Rich syndrome |
| 42040 | GP clinical event records | H563.12 | Cryptogenic fibrosing alveolitis |
| 42040 | GP clinical event records | H563.13 | Idiopathic pulmonary fibrosis |
| 42040 | GP clinical event records | H563z00 | Idiopathic fibrosing alveolitis NOS |
| 42040 | GP clinical event records | Hyu5000 | [X]Other interstitial pulmonary diseases with fibrosis |
| 42040 | GP clinical event records | X102v | Usual interstitial pneumonitis |
| 42040 | GP clinical event records | X102u | Pulmonary fibrosis |