Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic Pulmonary Fibrosis (IPF)

Patient Information

Respiratory Services

• Author ID:           HP 
• Leaflet Ref:         Med 015 
• Version:              2 
• Leaflet title:         Idiopathic Pulmonary Fibrosis (IPF)
• Date Produced:   January 2025 
• Expiry Date:        January 2027 

Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a condition in which the lungs become scarred/ stiff, resulting in breathlessness.

• Idiopathic (unknown cause)
• Pulmonary (Lungs)
• Fibrosis (scar tissue)

It is not clear what is the cause of the disease, however, it more commonly affects people who are around 70 to 75 years of age and over.

Signs and Symptoms

The symptoms of this disease tend to become more troublesome over time and can affect day to day life.

The symptoms which you may experience include:

• shortness of breath
• a persistent dry cough
• tiredness/fatigue
• loss of appetite and weight loss
• reduced exercise tolerance
• rounded and swollen fingertips (clubbed fingers)

What tests may I be sent for?

To obtain a diagnosis of IPF and to try to identify a possible cause of the disease, you may be sent for numerous tests that look at the function of the lung and how the lung is working.

These include:

• Chest X-Ray (CXR)
• High Resolution CT scan (HRCT)
• Pulmonary Function Test (PFT)
• Blood samples

Present Management Options

• Antifibrotic tablet medication (if appropriate)
• Symptom management 

Two antifibrotic medications (Nintedanib and Pirfenidone) have been identified as potentially reducing the rate at which IPF progresses (slowing the progression of scarring). However, these medications do come with many side effects.

If appropriate, the respiratory team will discuss these further with you.

Symptom Management 

The main aim of treatment for patients with IPF is early symptom management. This means, helping you with any day-to-day symptoms that are important to you.

These can be managed with medication, oxygen, and non-pharmacological strategies.

We work closely with the supportive and palliative care team. They will review your symptoms in a separate clinic appointment to identify your needs, and if appropriate, prescribe medication, or as suggested above, they will discuss non-pharmacological strategies to manage your symptoms. 

This referral may be offered to you if the clinician feels that it is appropriate.

Follow Up

You will be followed up routinely as decided by a respiratory team. This may be by a respiratory consultant or one of the specialist nurses. This could be face to face or via a phone consultation.

We do have a Community Support Group within the trust, which you are more than welcome to attend if you wish. Details are found below:

Last Thursday of every month from 2pm until 4pm.

Baden Powell Scout Hut, Turner Street, Wigan, WN1 3SU

IPF is a disease that there is no current cure for, and with this in mind, we are currently taking part in clinical trials in relation to IPF.

If you feel this would be something, you would like to consider later please let one of the team know. 

Please write any questions you may have here. These can be discussed at your next appointment.