What's idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis is a chronic, progressive scarring of the lungs of unknown cause. The scarring stiffens the lungs and makes breathing increasingly difficult.
| Also indexed as | ORPHA:2032, MONDO:0800504 |
|---|---|
| Features mapped | 18 |
| Treatments mapped | 5 |
| Published sources | 10 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Clubbing of fingers
Clubbing, a rounding and widening of the fingertips and nails, can develop in idiopathic pulmonary fibrosis and is sometimes noticed in the year before diagnosis.
Pulmonary fibrosis
IPF is a chronic, progressive scarring (fibrosis) of the lung tissue with no identifiable cause. The scarring builds up over time, stiffening the lungs and producing breathlessness, dry cough, and reduced breathing capacity.
Exertional dyspnea
Breathlessness on exertion is the main symptom and tends to worsen as the lung scarring progresses.
Honeycomb lung
'Honeycombing', clusters of cystic air spaces seen on CT scans, is a hallmark of the advanced lung scarring in IPF.
Subpleural honeycombing
On CT scans, clusters of small cystic air spaces known as honeycombing are a key feature of the lung scarring in idiopathic pulmonary fibrosis, typically near the lung surface.
Cough
A persistent dry cough is a common and often troublesome symptom.
Reticular pattern on pulmonary HRCT
On a high-resolution CT scan, IPF typically shows a net-like (reticular) pattern of scarring together with honeycombing, concentrated just under the surface of the lungs and in the lower zones. This distribution is the imaging signature used to diagnose IPF.
Bronchiectasis
Traction bronchiectasis, where scarring pulls the airways open into abnormal widened shapes, is a characteristic imaging feature of idiopathic pulmonary fibrosis.
Ground-glass opacification
On a chest CT scan, hazy areas called ground-glass opacities can appear. These have been seen alongside honeycombing and net-like (reticular) shadows during a flare-up (acute exacerbation) of idiopathic pulmonary fibrosis.
Crackles
Fine crackles that sound like Velcro being pulled apart are often heard at the lung bases through a stethoscope.
Decreased DLCO
Because scarring thickens the walls where oxygen passes into the blood, IPF lowers the lungs' diffusing capacity (DLCO) - a measure of how well gas crosses into the bloodstream. A low DLCO is common and tends to track with more advanced disease.
Usual interstitial pneumonia
The characteristic pattern on scans and biopsy is called usual interstitial pneumonia (UIP), which includes honeycomb-like changes in the lung.
Reduced forced vital capacity
Breathing tests in IPF show a restrictive pattern: the lungs become stiff and hold less air, so the forced vital capacity (the amount of air you can breathe out in one big breath) is reduced. Tracking FVC over time is a main way doctors follow how the disease is progressing.
Exercise intolerance
As idiopathic pulmonary fibrosis progresses, lung function falls and people become less able to tolerate physical activity.
Fatigue
Fatigue and a general sense of malaise are common in idiopathic pulmonary fibrosis and often precede the diagnosis.
Acute exacerbation
In idiopathic pulmonary fibrosis, an acute exacerbation is a sudden, severe worsening of the lung disease. It is one of the complications that can lead to early death.
Respiratory failure
As idiopathic pulmonary fibrosis progresses and lung function is lost, it can lead to respiratory failure. This is one of the complications that can shorten life.
Pulmonary hypertension
Pulmonary hypertension, raised blood pressure in the arteries of the lungs, can develop in idiopathic pulmonary fibrosis, where it is linked to a higher risk of death.
How it is diagnosed
Idiopathic pulmonary fibrosis
Diagnosed using: High-resolution CT (UIP pattern).
“Diagnosis of IPF necessitates confirmation of a 'usual interstitial pneumonia' (UIP) pattern on high-resolution computed tomography or on histology from a surgical lung biopsy.”
Idiopathic pulmonary fibrosis
Diagnosed using: Surgical lung biopsy.
“Diagnosis of IPF necessitates confirmation of a 'usual interstitial pneumonia' (UIP) pattern on high-resolution computed tomography or on histology from a surgical lung biopsy.”
Idiopathic pulmonary fibrosis
Diagnosed using: Multidisciplinary diagnosis (exclusion of other causes).
“…an IPF diagnosis can be made only after exclusion of any other cause for an interstitial lung disease.”
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
Nintedanib
Nintedanib is an antifibrotic medicine that can slow the decline in lung function in idiopathic pulmonary fibrosis.
Used to help with: Idiopathic pulmonary fibrosis.
“In particular, the shift from ineffective anti-inflammatory strategies to the success of nintedanib and pirfenidone in both IPF and non-IPF progressive diseases is discussed.”
Pirfenidone
Pirfenidone is an antifibrotic medicine that can slow the progression of idiopathic pulmonary fibrosis in people with mild to moderate disease.
Used to help with: Idiopathic pulmonary fibrosis.
“Based on the available evidence only pirfenidone has been shown to attenuate disease progression in mild to moderate IPF populations, with low-moderate quality of evidence.”
Long-term oxygen therapy
Long-term oxygen therapy is one of the supportive (non-drug) treatments used in idiopathic pulmonary fibrosis, alongside lung transplantation and pulmonary rehabilitation.
Used to help with: Idiopathic pulmonary fibrosis.
“Nonpharmacologic treatment options for IPF consist of long-term oxygen treatment, lung transplantation, and pulmonary rehabilitation.”
Lung transplantation
Lung transplantation is one of the non-drug treatment options in idiopathic pulmonary fibrosis, alongside long-term oxygen therapy and pulmonary rehabilitation.
Used to help with: Idiopathic pulmonary fibrosis.
“Nonpharmacologic treatment options for IPF consist of long-term oxygen treatment, lung transplantation, and pulmonary rehabilitation.”
Pulmonary rehabilitation
Pulmonary rehabilitation is one of the supportive (non-drug) treatments used in idiopathic pulmonary fibrosis, alongside long-term oxygen therapy and lung transplantation.
Used to help with: Idiopathic pulmonary fibrosis.
“Nonpharmacologic treatment options for IPF consist of long-term oxygen treatment, lung transplantation, and pulmonary rehabilitation.”
How to read the evidence labels
Where this comes from
This guide is built from 10 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
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