What is the Pulmonary Fibrosis?

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Pulmonary fibrosis is the process that takes place in a group of heterogeneous and chronic lung diseases. They are characterised by being rare, difficult to diagnose, and require complex treatments.    

Pulmonary fibrosis explained in first person

Professionals and patients explain how you live with the disease
What would be the future idea? It would be to revert the process of fibrosis, in other words, to be able to regenerate the damaged lung.
You have to take certain precautions and not take as many risks as before when going up or down; but, apart from that, if you take it easy, you can lead the life you want.

The fibrosing diffuse interstitial lung diseases (DILD) include a wide and diverse group of diseases with some characteristics in common. In all of them, the lung tissue is damaged and produces scars that gradually replace the healthy tissue, which leads to a worsening of symptoms and lung function.

Types of Pulmonary Fibrosis

Although the most known of the fibrosing DILD is idiopathic pulmonary fibrosis (IPF), there are other diseases that are similar, as they also present progressive pulmonary fibrosis.  

Depending on the origin of the disease, they can be classified into: 

Magnifying glass with question mark

Idiopathic. Fibrosis in which the cause is unknown, such as idiopathic pulmonary fibrosis (IPF) and idiopathic fibrosing non-specific interstitial pneumonia. 

Two lungs, one of them affected

Of unknown cause or associated with other diseases: 

  • Pneumonitis or pneumoconiosis due to chronic hypersensitivity.  

  • Fibrosing non-specific interstitial pneumonia associated with auto-immune diseases.  

  • Pneumonitis induced by drugs or radiotherapy.  

It is important to differentiate them from idiopathic pulmonary fibrosis, since the prognosis and treatment may vary depending on the disease. 

How many people are affected by Pulmonary Fibrosis?

The existing data on the incidence and prevalence of the fibrosing DILD vary widely. In Spain, 7.6 cases for every 100,000 inhabitants are diagnosed. The most common is idiopathic pulmonary fibrosis (IPF). It is estimated that there are currently between 7,500 and 10,000 patients in Spain with this disease. 

Substantiated information by:

Fernanda Hernandez Gonzalez
Jacobo Sellarés Torres
Joel Francesqui
Sandra Cuerpo Cardeñosa
Xavier Alsina Restoy

Published: 9 June 2020
Updated: 9 June 2020

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