AB FPI
Waterloo - About AB FPI

Avenue Reine Astrid 77 9
1410 Waterloo
3202354 46 06
     

What is idiopathic pulmonary fibrosis (IPF)?

Pulmonary fibrosis refers to the formation of scar tissue in the lungs. There are many different disorders, characterised by a combination of fibrosis and inflammation in varying degrees.

 

Although the types of pulmonary fibrosis have a known cause (inflammatory and auto-immune diseases or diseases resulting from high exposure to certain inhaled particles), the cause of idiopathic pulmonary fibrosis is by definition unknown.

 

With idiopathic pulmonary fibrosis, an excessive production of scar tissue irreversibly destroys the healthy lung structure. As the disease evolves, the lung function is impaired and oxygen absorption becomes increasingly difficult. Initially, the symptoms are only present during physical strain, but as the disease evolves, shortness of breath also manifests itself at rest. The disease generally appears between the ages of 50 and 70, more in men than in women and more in smokers or former smokers. Auscultation reveals the characteristic presence of crepitus redux and fairly characteristic deformation of the fingers is often observed (called digital hippocratism).

Why do some persons develop idiopathic pulmonary fibrosis?

As its name indicates, the cause of idiopathic pulmonary fibrosis is unknown. Experts currently believe that the development of IPF requires a combination of:

 

  • Particular setting (changes to the function of lung cells due to age or due to genetic changes, which explain certain family types).

  • Current or past exposure to elements which may irritate / damage the lungs (cigarette smoke, pollution, acidity due to gastro-oesophageal reflux, viral infections,...).

Once the lung cells have been damaged/irritated by these stimuli, excessive scar formation ensues because of the particular setting mentioned above. It is precisely this excessive scarring / repair process which is responsible for the development of fibrosis.

How to diagnose IPF?

It is difficult to diagnose pulmonary fibrosis; it requires a multidisciplinary team to analyse all the data, including:

  • An extremely detailed interview of the patient
  • A physical exam
  • Blood tests
  • A thoracic scanner
  • Lung function tests
  • Bronchoscopy and broncho-alveolar lavage

Sometimes a lung biopsy is necessary to confirm or invalidate the diagnose.

Treatments?

Two medicine-based treatments haven proven to be effective in slowing down the development of fibrosis. It concerns pirfenidone (Esbriet®) and nintedanib.

However, if the disease becomes too severe, oxygen must be administered and lung transplantation must be considered (in the absence of contraindications).

Numerous clinical studies are being undertaken and other treatments will hopefully become available in the coming years.

The Association’s goals are:

  • To inform and support patients suffering from idiopathic pulmonary fibrosis and their relatives

  • To organise moral support for patients and their relatives

  • To optimise patients’ access to care

 

Insofar as possible, to support actions improving the treatment and life quality of patients.

For more information and membership http://www.fibrosepulmonaire.be

 

E-mail : abfpi@mail.be

Company no.: 0556703685

Account no.: BE66 0017 3654 6843

BIC code: GEBABEBB

 

Association Belge contrela Fibrose Pulmonaire Idiopathique

 

Non-profit Association

 

F P I

 

Living with Pulmonary Fibrosis