[1 month, 1 rare disease, 1 organization] Idiopathic Pulmonary Fibrosis

Every month, BlueDil focus on a different rare and severe disease.


Rare disease of this month: Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a rare chronic, idiopathic lung disease characterized by progressive irreversible changes in the structure of the lungs. The proliferation of fibrosis-producing cells (consisting mainly of collagen) prevents the lung from functioning properly and the alveoli become rigid and retract.

The alveoli, those small pockets at the end of the bronchi where gas exchanges take place (absorption of oxygen and release of carbon dioxide).

The passage of oxygen and the release of carbon dioxide is no longer assured.

The term « idiopathic » means that the cause of this disease is unknown.


How is the disease manifested?

The initial symptoms of IPF are shortness of breath and often a chronic dry cough. It can also be signalled by recurrent infections, loss of appetite, unexplained weight loss, a feeling of fatigue and/or malaise, more rarely muscle and joint pain, and short, rapid breathing.

Digital hippocratism (enlargement and bulging of the last phalanges of the fingers or toes) is present in half of the cases. Early inspiratory crackles (crackling rales) are heard on auscultation.


Is there a cure?

There is no drug treatment that can cure IPF. The only thing that can be done is to slow its aggravation and the concomitant respiratory failure. Two anti-fibrotic drugs are currently available to slow down the progression of IVF and give a better quality of life to the patients concerned.

Another treatment – non-drug based – is recommended by pulmonary physicians: respiratory rehabilitation, which is provided in specialized hospital centers. It is useful to keep up appropriate physical activity, even if it is low.

Clinical trials are underway to test new molecules. In France, the coordination of care is ensured by the Reference Centre and the Competence Centres for Rare Lung Diseases (www.maladies-pulmonaires-rares.fr) with which lung specialists work.


For more information, visit APEFPI‘s website