idiopathic pulmonary fibrosis
Findings
No curated finding names idiopathic pulmonary fibrosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
An interstitial lung disease with a poor prognosis, that is characterized by the progressive formation of scar tissue within the lungs in the absence of any known cause.
Definition from the Mondo Disease Ontology (MONDO:0800504), read 2026-09-29. CC BY 4.0.
Features
20 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal pulmonary interstitial morphologyHPOHP:0006530
- Very frequent (80% to 99% of cases)
- AcrocyanosisHPOHP:0001063
- Frequent (30% to 79% of cases)
- BronchiectasisHPOHP:0002110
- Frequent (30% to 79% of cases)
- Clubbing of fingersHPOHP:0100759
- Frequent (30% to 79% of cases)
- CoughHPOHP:0012735
- Frequent (30% to 79% of cases)
- CracklesHPOHP:0030830
- Frequent (30% to 79% of cases)
- Decreased DLCOHPOHP:0045051
- Frequent (30% to 79% of cases)
- Exertional dyspneaHPOHP:0002875
- Frequent (30% to 79% of cases)
- FatigueHPOHP:0012378
- Frequent (30% to 79% of cases)
- Gastroesophageal refluxHPOHP:0002020
- Frequent (30% to 79% of cases)
- Ground-glass opacificationHPOHP:0025179
- Frequent (30% to 79% of cases)
- Honeycomb lungHPOHP:0025175
- Frequent (30% to 79% of cases)
Show the remaining 8
- Pulmonary fibrosisHPOHP:0002206
- Frequent (30% to 79% of cases)
- Reduced forced vital capacityHPOHP:0032341
- Frequent (30% to 79% of cases)
- Reticular pattern on pulmonary HRCTHPOHP:0025390
- Frequent (30% to 79% of cases)
- Subpleural honeycombingHPOHP:0031631
- Frequent (30% to 79% of cases)
- Usual interstitial pneumoniaHPOHP:0031950
- Frequent (30% to 79% of cases)
- Exercise intoleranceHPOHP:0003546
- Occasional (5% to 29% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- ELMOD2HGNC:28111
- No Known Disease Relationship · ClinGen · Unknown · 2025
Where it sits
Other names
1 name
Resolves to: idiopathic pulmonary fibrosis
- Also called
- IPF