Idiopathic pulmonary fibrosis
Fibrosis pulmonum idiopathica · Idiopatická plicní fibróza
What it is
Lung tissue scars progressively; stiff lungs cannot expand.
Symptoms & signs
- Dry cough
- breathlessness on exertion
- finger clubbing
- velcro crackles
- weight loss
🚩 Get help if…
- call 112 / 911 now Breathlessness getting sharply worse over days
- call 112 / 911 now Blue lips, or breathless at rest
- see a doctor soon Fever with cough and more breathlessness
- see a doctor soon Swollen ankles, or needing more oxygen
Where it strikes
Medical treatment
- Pirfenidone or nintedanib slow decline
- oxygen
- pulmonary rehabilitation
- lung transplant
Natural & lifestyle
- Reflux control
- vaccination
- exercise training
- avoid dust and bird exposures
Emerging & experimental
- Anti-fibrotic combinations
- LPA1 antagonists
- senolytics
- stem-cell and lung bioengineering
Alternative, off-label & anecdotal
Approaches used outside the guidelines, with what their supporters report and what testing has found so far.
- N-acetylcysteine alone — the PANTHER trial found no benefit; a genetic subgroup is being tested again not confirmed in trials
- Senolytics (dasatinib + quercetin) — a small pilot study improved walking distance early / small studies
- Serrapeptase and curcumin — sold as 'dissolving scar tissue'; no human evidence anecdotal / traditional
- Singing and breathing-muscle training — small trials show less breathlessness early / small studies
ICD codes
- ICD-10 J84.1
- ICD-11 CB03.4 Idiopathic pulmonary fibrosis