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Pulmonary Fibrosis

Pulmonary fibrosis (PF) is the end of result of many different conditions that cause scar tissue to build up in your lungs and make breathing increasingly difficult as your lungs become stiffer and less elastic, so they are less able to move and take oxygen from the air you breathe.

Interstitial Lung Disease (ILD) causes scarring in your lungs, inflammation in your lungs or a mix of both. Some diseases cause more of one or the other. It is important to know which the major cause of your symptoms is to help decide the best treatment for you. The treatment and outlook for different types of pulmonary fibrosis vary considerably.
 

  • All types of pulmonary fibrosis are considered rare.
  • Pulmonary Fibrosis is an interstitial lung disease (ILD). There are more than 200 different ILDs.
  • Pulmonary – it affects your lungs.
  • Fibrosis – a build up of scar tissue, which makes your lungs stiff.
  • Interstitial – affects your interstitium, the network of tissue that support air sacs in your lungs.

 

The cause of pulmonary fibrosis can be identified in some cases, but for many types a definite cause cannot be found. Some of the causes are:

  • Exposure to certain types of dust – including wood or metal dusts or asbestos.
  • Exposure to allergens carried in the air – such as bird feathers or mould.
  • A side effect of a drug
  • Some types of Pulmonary fibrosis are more likely to occur when you have another condition such as rheumatoid arthritis or scleroderma. These are sometimes called connective tissue disease related ILDs or CT-ILD

In very few cases, two or more members of the family may develop pulmonary fibrosis. Current UK data suggests that less than 10% of people with pulmonary fibrosis have inheritable disease.


Symptoms
  • Shortness of breath on exertion, such as climbing a hill or stairs, however you may also feel out of breath when not moving around as much.
  • Constant cough
  • Feeling tired all the time

Some people can also loose weight, develop a fever, aching, stiff joints and muscles, rashes, dry mouth and eyes, or poor circulation in fingers and toes (Raynaud’s)


How do we diagnose PF?

Your doctor will examine you, looking for other causes of breathlessness. They will listen for crackles in your lungs and arrange a chest x-ray if they hear any. If there is a suspicion, you may have lung fibrosis you will be referred to a chest specialist.

You will be asked questions about your medical, family and work history and your symptoms.
 

Tests
  • CT Scan of your lungs
  • Breathing and lung function tests to measure how well your lungs are working.
  • Blood tests


Your doctor may need to look inside your lungs and possibly remove some cells or tissue for testing. Different procedures can be used to get some lung tissue.

  • Flexible bronchoscopy
  • Video assisted thoracoscopy (VATS)

 

Treatment
  • Drug treatments – the aim is to slow down the rate of scarring in the lungs.
  • Steroids or other immunosuppressant drugs are used to supress the inflammation.
  • Lung Transplant

 

Look after yourself
  • Have a flu jab each year.
  • Get your one-off pneumonia vaccination.
  • Stay as fit as you can.
  • Eat a healthy, balanced diet and maintain a healthy weight.
  • Try different techniques and positions to help your breathing.