Building a stronger voice for IPF

What is Idiopathic Pulmonary Fibrosis?

“Idiopathic” means unknown – no one knows the exact cause of IPF. What is understood is that, with IPF, there is a change in the lung’s normal healing process that produces extra scar tissue. Unfortunately, there are no symptoms of this change until the scar tissue builds up in the lungs and affects breathing. The causes of this abnormal healing process are unknown, but there are a number of factors that may be associated with an increased risk of developing IPF.

Potential risk factors associated with IPF

Cigarette smoking may increase the risk of developing IPF. Some cases of IPF occur in patients who have family members with IPF, which suggests that genetics may contribute to the risk of developing IPF in certain individuals. Although these risk factors are associated with IPF, it is important to remember that they have not been shown to cause IPF − the cause of the disease is still unknown.

The incidence of IPF

The precise incidence and prevalence of IPF is difficult to determine, however it is a relatively rare disease. Based on several sources it is estimated that there will be approximately 30,000–35,000 new patients diagnosed with IPF in the 27 EU countries each year. More cases generally occur in men than in women and it is usually diagnosed in adults who are aged between 40 and 80 years old.

 

What are the symptoms of IPF?

– Early signs and symptoms typically include:

 

  • Chronic cough: usually a dry cough that doesn’t produce phlegm or mucus.
  • Shortness of breath that occurs while performing certain physical activities, particularly those that have not previously caused this symptom.

– Late signs and symptoms of IPF may include:

  • Shortness of breath and/or episodes of coughing, while at rest, that affect a person’s ability to eat, talk on the telephone, or walk up and down steps.
  • Enlargement of the fingertips, and thickening of the nail beds with a spoon-shaped appearance called “clubbing” occurs in some patients. Patients may also experience fluid retention in the legs and arms, this is known as peripheral oedema.

– When should one go to the doctor?

    • For many patients it can take up to two years from the appearance of initial symptoms − usually a dry, non-productive cough − for IPF to be first diagnosed. You should, therefore, consult a doctor if you experience any of the symptoms described above, to find out what might be causing them. These symptoms are quite general and many types of illness could cause them, not solely IPF.
    • IPF may be difficult to diagnose for a number of reasons. Its symptoms are similar to those of other illnesses, such as asthma, chronic obstructive pulmonary disease (COPD), and congestive heart failure. IPF can also co-exist with other lung diseases, including asthma and COPD. In order to confirm a diagnosis of IPF, physicians must rule out all known causes of fibrotic lung disease; it often takes time, therefore, to explore and eliminate other possible diagnoses.

Progression

How does IPF progress?

The clinical course differs from patient to patient and cannot be readily predicted even when the diagnosis has been made. For some patients, the illness and its symptoms can remain relatively stable for years, whereas with other patients the symptoms can worsen rather quickly. A unique aspect of IPF is that the medical condition can deteriorate in sudden, unexpected jumps − doctors speak of “acute exacerbations”. These IPF exacerbations are marked by an intense shortness of breath, violent coughing episodes and fatigue.

Mechanism of the disease

How does IPF arise?

IPF is a specific type of pulmonary fibrosis in which the small air sacs of the lung, known as “alveoli”, gradually become replaced by fibrotic (scar) tissue. The scarring begins in the tissue between the air sacs. Normally, this tissue is composed of a thin layer of cells, but scarring with IPF causes the tissue to thicken and become stiff, preventing oxygen from getting into the bloodstream. Over time, this scarring of the lungs worsens, the lungs become stiffer and breathing becomes more difficult. A person will find that they become increasingly short of breath. The actual cause of IPF is currently unknown. This distinguishes IPF from other forms of lung fibrosis for which a definite cause is known and better understood, such as exposure to a harmful substance like asbestos.

Diagnosis

How is the diagnosis made?

There are many types of lung disease that can produce symptoms similar to those of IPF. For this reason several tests are used to make a diagnosis − specialists in diseases of the lungs, known as pneumologists, may perform some of these tests.

Physical examination

The majority of patients who are eventually diagnosed with IPF first visit their doctor because they have a dry cough or shortness of breath. Initially a physical examination is performed. When listening to the lungs (auscultation), the doctor can often hear a crackling sound in both lungs at the end of inhalation − a sound like the one made by opening a VELCRO® fastener. A lack of oxygen in the bloodstream can lead to a violet or bluish coloration of the skin and fingernails. Additionally, around one-half of patients with IPF will eventually have thickened fingertips (clubbed fingers) and enlarged, spoon-shaped fingernails (“hippocratic” nails).

Imaging procedures

Chest X-rays

It is harder for X-rays to pass through fibrotic lung tissue than through healthy lungs. The changes in lung tissue that are seen in IPF patients become visible on X-ray images as white areas in the places where the X-ray beam is reflected away, instead of easily passing through the lung itself. These thickened white areas cannot be easily seen in the X-ray pictures of all patients and, once observed, a radiologist may want to study them in greater detail using more sophisticated techniques, such as a high resolution computer tomography (HRCT) scan.

High resolution computer tomography (HRCT) scan

HRCT scans of the chest are very precise and they make it possible to recognize IPF disease, as well as other chest abnormalities, at an earlier stage. Computer tomography pictures can be taken in many, very thin, slices and this allows for highly detailed and accurate mapping of the chest area. This method ensures that any changes or abnormalities will be recognized with greater accuracy. The HRCT images of patients with idiopathic lung fibrosis typically appear with “open spaces” inside the lung, with areas along the edges of the chest wall that look like honeycombs.

Lung function tests

Lung function tests are performed to measure a person’s breathing capacity, to determine the “stiffness” of the lungs, and to calculate the amount of oxygen that transfers from the lungs into the bloodstream. These can be done easily, and are very accurate ways of diagnosing current lung function, but they can also be used to follow a patient’s condition over time. These are non-invasive tests.

Bronchoscopy (lung endoscopy)

A bronchoscope is a flexible tube with a camera attached to the end that a pneumologist uses to visually examine the airways. This examination is usually conducted with sedation and is not painful. The bronchoscope is passed through the patient’s nose or mouth and advanced into the airway to look at the anatomy and, occasionally, to collect samples of lung tissue, fluid or foreign material, such as infections. With the bronchoscope it is possible to obtain a specimen of secretions from the lungs so that these can be examined microscopically and microbiologically

Biopsy

If the above tests do not lead to a clear diagnosis for the patient, a surgical procedure can be performed to take further samples of lung tissue for subsequent examination under a microscope. In virtually all cases, this will allow a further, detailed assessment of the disease, and can result in a reliable diagnosis. Today, this procedure can almost always be performed by means of gentle, keyhole technology using a video-assisted thoracoscopy (VATS). This procedure is performed under general anaesthesia and is the least invasive way of taking a biopsy of the lungs.

Therapy

Talk with your doctor, who will help you to decide which treatment is most appropriate for you. If you are prescribed a therapy, it is important to take your medicine exactly as prescribed. Your doctor will want to see you on a regular basis to check on how you are doing. In addition, there are non-drug therapies that can help you to manage your symptoms. There are various ways to treat IPF and alleviate its symptoms. These include medications, some procedures that can assist with breathing and lung transplantation.

Lung transplantation

A potential treatment for IPF is lung transplantation. This option can only be considered for a few patients. It is difficult to find suitable donor organs, and patients must be in a sufficiently healthy physical condition to undergo a transplant procedure. The upper age limit is currently 65 years at many centers. However, there are many factors that need to be considered before this is recommended. Your doctor can discuss these with you.

Associated conditions

GERD (Gastro-Esophageal Reflux Disease):

Many patients experience heartburn, or even painless reflux of acid into the throat (also referred to as asymptomatic GERD). This is often treated with medications that suppress production of acid in the patient’s stomach.

Self-management

What can YOU do?

Patients affected with IPF can do a lot to favorably influence the course of the disease. We would like to introduce these measures to you.

Stop smoking

Smoking is a known risk factor for causing IPF. Disease progression is accelerated by smoking. All patients can therefore benefit by quitting smoking. Furthermore, smoky rooms and air contamination of all types should be avoided.

Pulmonary rehabilitation

Pulmonary rehabilitation programs consist of exercise training, breathing techniques, medical education, nutrition advice and emotional support provided by a team of nurses, respiratory and physical therapists, social workers, and dieticians. Speak to your physician about enrolling in a pulmonary rehabilitation program.

Nutrition

Balanced nutrition and healthy eating is important for overall health and well being. Patients with IPF should try to achieve or maintain a normal body weight. Being overweight causes unnecessary stress on the body and can increase breathing difficulties. In contrast, being underweight can make it harder for your body to defend itself against infection, and should also be avoided.

Vaccinations

IPF patients often suffer from respiratory tract infections. Therefore, older patients should be sure to have regular vaccinations. A vaccination against the pneumonia (pneumococcus) infection is worth considering, as is a flu shot.