About Pulmonary Fibrosis

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1 About Pulmonary Fibrosis Being diagnosed with pulmonary fibrosis may be a frightening experience. As it is an uncommon form of lung disease, many people feel isolated and are unable to find helpful and accurate information about pulmonary fibrosis. At, we want to support people with pulmonary fibrosis as well as their carer s, family members and friends. On this website we have provided up to date information about pulmonary fibrosis which we hope is helpful and informative. What is Pulmonary Fibrosis? Pulmonary fibrosis is a term that describes the formation of scar tissue within the lungs. It usually occurs around the small air sacs or alveoli of the lungs. The alveoli are important for the transfer of oxygen and carbon dioxide from the lungs into the bloodstream. Over time, the scar tissue builds up around the alveoli, making it harder for oxygen to pass from the lungs to the body where it is needed. This results in feeling short of breath. At first, this may occur with more strenuous activities such as walking uphill or stairs. However as the fibrosis increases, you may notice you are breathless with all activities, including washing and dressing, eating and talking. 1

2 Causes There are over 200 different types of pulmonary fibrosis. For instance pulmonary fibrosis can occur in association with a number of autoimmune or connective tissue diseases such as Rheumatoid Arthritis, Systemic Lupus Erythematosus (SLE), Systemic Sclerosis and Sjogren s syndrome. It can also result from certain occupational exposures, including asbestos which is known as asbestosis; as well as coal dust and/or silica which are called pneumoconiosis. In addition, some medications may cause lung fibrosis, such as amiodarone, nitrofurantoin and bleomycin. Pulmonary fibrosis may also occur following inhalation of irritant substances as with hypersensitivity pneumonitis that is commonly caused by exposure to birds or moulds. However for a number of people, the cause of their pulmonary fibrosis cannot be identified and for this reason it is known as idiopathic pulmonary fibrosis or IPF. Although pulmonary fibrosis is rare, the symptoms are similar to many more common lung diseases, such as asthma or chronic obstructive pulmonary disease (COPD). This makes them difficult to diagnose. 2

3 Diagnosis As there are so many different types of pulmonary fibrosis, it is very important to determine what kind of pulmonary fibrosis you have. In order to do this, your hospital doctor will want to ask about your breathing problems, perform an examination and a number of tests. These results are discussed by respiratory physicians, radiologists, thoracic surgeons and pathologists who specialise in pulmonary fibrosis and other interstitial lung diseases in a multi-disciplinary meeting (or MDT). Your hospital doctor will discuss these results with you to provide you with the most accurate information about your lung disease and the best treatment options available. What is Idiopathic Pulmonary Fibrosis or IPF? Idiopathic pulmonary fibrosis (IPF) is the commonest form of pulmonary fibrosis. As its name suggests, the cause of this lung disease is unknown. The condition used to be called cryptogenic fibrosing alveolitis or CFA. It is sometimes referred to as usual interstitial pneumonia (UIP). IPF is a progressive, scarring lung disease that has a very poor outcome. The middle and lower parts of the lungs are affected first. As IPF progresses, the lungs slowly become much stiffer and develop a honeycomb appearance. In some people the scarring develops very quickly, whilst in others it happens over a longer period of time. On average, an individual with IPF has a life expectancy of 3 years. Who gets IPF? IPF usually affects people over the age of 60 years and is more common in men than women. Studies have demonstrated the IPF is more common in people who smoke and in association with certain occupations. Occasionally it can occur in younger people, especially if there is a family history of pulmonary fibrosis. In the UK, there are approximately 5000 new cases of IPF each year. The number of cases of IPF is increasing not only in the UK, but also in the USA and Europe. We do not understand why this is happening. What are the symptoms of IPF? The most common symptoms are: Shortness of breath in early IPF, this may occur with more strenuous exercise such as walking uphill or stairs. However as the disease progresses, you may notice breathlessness with other activities such as washing and dressing, talking and eating. Cough this is usually a dry, tickly cough. Some people with IPF may have a cough productive of phlegm. Fatigue Unintentional weight loss 3

4 What are the signs of IPF? Clubbing this is the widening and rounding of the fingers or toes. This occurs in up to 60% of people with IPF Velcro-like crackles these are usually heard in the lower part of the lungs on examining the chest How is IPF diagnosed? Your hospital doctor may ask you about the following: Your symptoms cough and breathlessness Your occupations, particularly jobs where you may have been exposed to asbestos, wood or stone dust, metals Your hobbies and pets If you have ever smoked A family history of lung disease Details of prescribed and over-the-counter medications Your health in general Your doctor will perform a physical examination and you may have a number of tests including the following: Blood and urine tests Lung or pulmonary function tests Body Plethysmography chamber Body Plethysmography chamber In particular, these tests measure your lung capacity (forced vital capacity or FVC) and how efficiently the lungs transfer gases to and from the blood (gas transfer or TLCO). Assessment of oxygen saturation A probe or pulse oximeter will measure your oxygen saturation at rest and with exercise, such as during a 6 minute walk test or shuttle test. The probe is placed either on the toe or fingertip. Echocardiogram This is an ultrasound scan that assesses the function of the heart. In particular, it is used to look for increased pressure on the right side of the heart, which is called pulmonary hypertension. 4

5 Chest x-ray In individuals with IPF, the scarring usually starts in the lower part of the lungs and leads to a reduction in lung volume. CT scan A CT scan provides a highly detailed three-dimensional picture of the lungs. In IPF, the typical pattern of fibrosis is described as honeycombing. However, approximately 20-30% of people with IPF do not have these typical CT appearances and require additional investigations, such as bronchoscopy or lung biopsy, to make a diagnosis of IPF. Bronchoscopy This procedure samples the cells within the air sacs or alveoli. A small camera (bronchoscope) is passed either through the nose or mouth into the lungs. It is performed with local anaesthetic and a sedative. Lung biopsy In some circumstances, a biopsy of the lung is required. This is usually performed by a keyhole method called video assisted thorascopic surgery or VATS. This will be discussed with you by your hospital specialist and a thoracic surgeon. 5

6 Treatment What treatments are available? At present, there are no treatments that reverse or cure IPF. But there are newer treatments available that may help to slow down the rate of progression of the lung fibrosis. Please discuss these treatments with your hospital doctor. Nintedanib Nintedanib is a targeted therapy which prevents that action of growth factors which may be important in the progression of pulmonary fibrosis. The IMPULSIS studies showed beneficial effects of Nintedanib on lung function in patients with mild to moderate IPF. This drug is not yet available for general use within the UK. However, on the basis of the data in the IMPULSIS studies it is possible that nintedanib will get approval in the near future. Your local ILD physician will be informed as soon as it is available in the UK. Pirfenidone This is the only licenced treatment for IPF. In clinical trials, it has been shown to reduce the rate of decline in lung function (FVC and TLCO) in people with mild to moderate IPF. It has now been approved by NICE for individuals with mild to moderate IPF, according to lung function criteria. For further information on pirfenidone, please click here to download a PDF by the National Institute for Health and Care Excellence. The ASCEND study has just reported further beneficial effect of Pirfenidone on lung function in patients with mild to moderate IPF. This is extremely encouraging. N-acetylcysteine Healthy lungs contain substances called anti-oxidants that help to protect the lungs from damage, often caused by toxic substances known as oxidants. In IPF, there is an imbalance between the level of anti-oxidants and oxidants. N- acetylcysteine (often called NAC) is an antioxidant therapy that may be helpful for some people with IPF. The Panther study has recently reported that in patients with very mild IPF there is no improvement in lung function or survival when using NAC, however there was some suggestion that it may help with symptoms. Furthermore, it is not possible to say whether or not there would be a beneficial effect in patients with moderate or severe IPF. 6

7 Acid reflux therapy Gastro-oesophageal or acid reflux is common in people with IPF. There are a number of medical and surgical treatments available to control acid reflux. Clinical trials Over the last 10 years there has been an enormous growth in research into understanding the cause and mechanisms that are responsible for IPF. This is a very exciting time as it has identified new molecules that may be involved in the disease process and could lead to new drug therapies in the future. As a result of this research, there has been an increase in the number of clinical trials in IPF. In order to determine if a drug is going to be effective, it has to be compared to a substance which is not known to have any effect, called a placebo. The trial has to be conducted in a double blind manner to ensure that the outcomes are not biased. This means that neither the person taking part nor their doctor know what treatment is being given. People taking part in clinical trials are closely monitored for any side effects. In the UK, there are a number of centres which take part in clinical trials for IPF. If you would be interested in taking part in a clinical trial, please discuss this with your hospital specialist doctor who will be able to advise. Further details about current IPF clinical trials can be found on the website clinicaltrials.gov Oxygen Therapy If your oxygen levels are low, your doctor may discuss oxygen treatment. Ambulatory oxygen this is given to people who is oxygen levels are low with exercise Long-term oxygen therapy (LTOT) this is given to people who is oxygen level is low at rest as well as with exercise. Pulmonary rehabilitation Pulmonary rehabilitation was initially established for people with COPD, however many patients with lung fibrosis benefit from this program. It consists of a six week course which provides advice on: How to manage breathlessness Exercises to improve muscle strength and fitness Self-management training Psychosocial support 7