What it is
Idiopathic pulmonary fibrosis is a chronic, progressive scarring of the lungs of unknown cause. The scarring stiffens the lungs and makes breathing increasingly difficult.
Signs and symptoms
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.
Cough
A persistent dry cough is a common and often troublesome symptom.
Crackles
Fine crackles that sound like Velcro being pulled apart are often heard at the lung bases through a stethoscope.
Usual interstitial pneumonia
The characteristic pattern on scans and biopsy is called usual interstitial pneumonia (UIP), which includes honeycomb-like changes in the lung.
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.”
Turn this into questions for your doctor
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How to read the evidence labels
Where this comes from
This guide is built from 2 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.