Idiopathic pulmonary fibrosis (IPF)
A severe progressive lung disease
Dr. Karim Benhaddou
Pulmonologist
In simple terms
Pirfenidone, nintedanib, transplant: slowing IPF progression.
Progressive lung scarring
IPF is a chronic interstitial pneumonia of unknown cause. Mainly affects men >60 years. Median survival without treatment: 3-5 years.
Symptoms
Progressive exertional dyspnea, dry cough, Velcro-like crackles on auscultation, clubbing.
Diagnosis
High-resolution chest CT (typical UIP pattern), pulmonary function tests (restrictive pattern, decreased DLCO), multidisciplinary discussion. Lung biopsy in selected cases.
Treatments
- Pirfenidone, nintedanib: antifibrotics, slow functional decline
- Long-term oxygen if hypoxemia
- Pulmonary rehabilitation: improves quality of life
- Lung transplant: only curative option for eligible patients
Management
Vaccinations (flu, pneumococcus, COVID), avoid respiratory infections, supportive care, palliative care.
The words explained
Medical terms used above, in everyday language.
- Chronic
- A problem that lasts a long time, usually more than three months, and often comes back.
- Symptom
- A sign you can feel or see that something is wrong, like pain, tiredness or a rash.
- Diagnosis
- The moment a doctor identifies which illness is causing your symptoms.
- Vaccine
- A safe training session for your immune system, so it can fight a germ later.
When should you see a doctor?
- Your symptoms last more than a few weeks or keep coming back.
- They get worse quickly, or stop you doing everyday things.
- You notice sudden pain, breathing trouble, fainting or bleeding — seek urgent care.
- You already take medication and want to change anything about it.
This article explains general health information. It does not replace advice from your own doctor.
Frequently asked questions
- What is Idiopathic pulmonary fibrosis (IPF)?
- Pirfenidone, nintedanib, transplant: slowing IPF progression.
- What are the main symptoms or signs of Idiopathic pulmonary fibrosis (IPF)?
- Common signs vary from person to person. The article above lists the symptoms to watch for, how they progress, and when they need medical attention.
- When should you see a doctor for Idiopathic pulmonary fibrosis (IPF)?
- See a doctor if symptoms last more than a few weeks, get worse quickly, stop you doing everyday things, or if you notice sudden pain, breathing trouble, fainting or bleeding.
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