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Conditions with pulmonary fibrosis

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a medical term. Pulmonary refers to the lung. Fibrosis is another word for scarring. And idiopathic means that the cause is unknown. All together, IPF means that you have scarring on your lungs from an unknown cause.1,2

Common signs and symptoms of idiopathic pulmonary fibrosis

Most people don’t notice the changes to their lungs at first and may think they’re unfit or tired. This can be true but it’s important to be aware of your body’s capabilities and notice any changes, especially if you have any risk factors for developing IPF.

Idiopathic pulmonary fibrosis
Cough puff
Cough1,2

Cough—usually a dry one—is the most common symptom and may start long before your disease is diagnosed

Shortness of breath
Shortness of breath1,2

This can happen with physical activity and develops as the disease gets worse

Quick tip

Keep a record of any symptoms you may have been experiencing, how often, how long they last, and whether they make it harder for you to do things. This symptom tracker can help you stay organised.

Risk factors for IPF

Although the cause of IPF is unknown, certain factors may increase the risk, such as3:

Cigarette smoking
Cigarette smoking
Virus
Some viral infections
DNA Icon
Genetics
Stomach
Gastro-oesophageal reflux disease (GERD)

If you have risk factors for developing IPF or are experiencing symptoms of IPF, ask your doctor for a lung examination as soon as you can.

Diagnosing IPF

The process of diagnosis will likely include multiple tests and visits with several types of doctors. Your treatment team will look at many areas of your health. For example, they will ask you about your work history, family medical history, medications that you are taking, and whether you smoke or have ever smoked.4

Recognising IPF early is key to getting the help you need. Ask your doctor for a lung examination, it can give your doctor a better understanding of your lung health. In these examinations, your doctor may:

stethoscope
Listen with a stethoscope for crackling sounds in the lungs5
CT Scan
Conduct breathing tests or administer a computed tomography, or CT, scan6
Finger
Look for finger clubbing (widening and rounding of the tips of fingers or toes, with a spongy sensation)5

However, it is important to remember that only a high-resolution computed tomography (HRCT) scan can provide a definitive diagnosis.6

Prognosis and treatment for IPF

Prognosis and treatment for IPF

Some research suggested that half of people with IPF live for about 3 to 5 years after diagnosis. However, these estimates were based on information provided before new treatments for IPF were available.1,2

With new antifibrotic treatments available to them, people with IPF can live longer. Although the new treatments cannot reverse the scarring on the lungs, they may slow the progression of the disease.7

Monitoring the progression of IPF

IPF is a progressive disease, which means that it gets worse over time. However, there is no way of predicting how fast this will happen. Unlike many other chronic diseases, there are no clear-cut ‘stages’ of IPF. Some patients may notice few changes in their symptoms, while other patients may experience a faster worsening of their symptoms. It is not known why the disease progresses faster for some people than others.8,9

It’s important that your healthcare professional checks your lung health regularly so that any changes can be managed quickly.10 Your doctor, supported by a wider treatment team of experienced specialists, will:

  • Review your medical history
  • Conduct a physical examination
  • Look for progression of your disease by administering tests you may have already had when you were diagnosed 
  • Discuss treatment options and tailor your treatment plan

Sudden, rapid worsening of symptoms

In the early stages of IPF, you may only notice small changes in your breathing. As the disease progresses, there is a bigger risk of complications or an acute exacerbation occurring. An acute exacerbation is a sudden and rapid worsening of symptoms.9

If you have a fever, you may think you have a chest infection or are experiencing a flare-up of your condition, but it's important you seek medical advice anyway. Acute exacerbations can be life-threatening, and for this reason, it is vital that you work with your treatment team to do everything you can to look after your lungs.9

Alert icon
Important

If your symptoms worsen suddenly, keep calm and alert someone nearby so they can contact your treatment team. If your symptoms are severe, you or someone close to you should contact the emergency services.

Managing life with IPF

Receiving a diagnosis of IPF can make you feel lonely and uncertain; at these times it can feel very difficult to stay positive. Daily life can be challenging, both practically and emotionally. However, there are many strategies to help people living with pulmonary fibrosis.

Managing life with IPF

Explore treatment options for people with idiopathic pulmonary fibrosis.

Learn more

Abbreviations: CT, computed tomography; GERD, Gastro-oesophageal reflux disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; PF, pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease.

  1. Martinez F, Collard H, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.

  2. Meltzer E, Noble P. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.

  3. Fernandez I, Eickelberg O. New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. Lancet. 2012;380(9842):680-688.

  4. Asthma + Lung UK. How is pulmonary fibrosis diagnosed? Updated June 2, 2022. Accessed October 2, 2024. https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/how-pulmonary-fibrosis-diagnosed

  5. Wells AU, et al. Interstitial lung disease guideline. Thorax. 2008;63:v1–v58.

  6. Asthma + Lung UK. What are the symptoms of pulmonary fibrosis? Updated June 2, 2022. Accessed October 2, 2024. https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/symptoms

  7. Woodcock H, Maher T. The treatment of idiopathic pulmonary fibrosis. F1000Prime Rep. 2014;6:16.

  8. American Lung Association. Stages of pulmonary fibrosis. Updated June 7, 2024. Accessed October 2, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/stages-of-pulmonary-fibrosis

  9. Kim DS. Acute exacerbations in patients with idiopathic pulmonary fibrosis. Respir Res. 2013;14(1):86.

  10. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.