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Start free with EleplanIdiopathic pulmonary fibrosis
ORPHA:2032Disease
Also called IPF
What it is
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.
Key facts
- Prevalence
- 1-5 / 10 000
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
16- Acrocyanosis
- Bronchiectasis
- Clubbing of fingers
- Cough
- Crackles
- Decreased DLCO
- Exertional dyspnea
- Fatigue
- Gastroesophageal reflux
- Ground-glass opacification on pulmonary HRCT
- Honeycomb lungDiagnostic criterion
- Pulmonary fibrosisDiagnostic criterion
- Reduced forced vital capacity
- Reticular pattern on pulmonary HRCTDiagnostic criterion
- Subpleural honeycombing
- Usual interstitial pneumoniaDiagnostic criterion
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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