Idiopathic pulmonary fibrosis

in patients over 50 years old, the disease is more frequent

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Diagnosis and Treatment at the Clínica

The diagnosis is made using a clinical examination, chest x-ray and CT scan [SP].

A pulmonary tissue biopsy [SP] is done to rule out any other known causes of interstitial pulmonary diseases, such as the toxicity in certain drugs, environmental exposures or collagen tissue disorders like systemic lupus erythematosus [SP] or systemic sclerosis [SP].

The bronchoalveolar lavage carried out by fiberbronchoscopy helps to rule out other diseases but is less specific in its diagnosis of pulmonary fibrosis.

In the pulmonary function tests [SP] there are some characteristics that suggest pulmonary restriction or capacity problems when exchanging gases (oxygen and carbon dioxide).

The conventional treatment consists in corticoids, immunosuppressive agents/ cytotoxics and antifibrotic agents.

Oxygen is sometimes necessary for patients who have low oxygen levels in their blood.

Pulmonary rehabilitation can help to maintain their ability to do exercise without breathing difficulties.

Some patients require a pulmonary transplant. This procedure is usually considered for patients under 65 years old whose disease has not been responding well to treatment.


Do you want to learn more about the Department of Pneumology?

We evaluate patients with pulmonary fibrosis from a multidisciplinary point of view, working with the Departments of Radiology, Pathology and Thoracic Surgery, etc.

Generally, idiopathic pulmonary fibrosis progresses over time, although the course of the disease varies: some people remain stable over several years and others get worse very quickly, in just a few months.”

Learn more about idiopathic pulmonary fibrosis

Idiopathic Pulmonary Fibrosis is a chronic disease which affects both lungs diffusely.

It is characterised by a progressive scarring (fibrosis) that means that the lung loses its flexibility, and its long term functioning deteriorates. 

It’s an interstitial pulmonary disease and currently four known types of the disease have been classified through biopsies, these conditions are also referred to as interstitial pneumonias:

  • Usual interstitial pneumonitis
  • Desquamative interstitial pneumonia 
  • Acute interstitial pneumonia
  • Non-specific interstitial pneumonia

The cause is unknown.

The potential risks of developing this disease are as follows:

  • Tobacco: it seems that there is a direct link between this disease and the number of cigarettes smoked a day over several years
  • Medication usage: a connection has been found between this disease and certain antidepressants
  • Chronic gastric suction: secondary to a gastroesophageal reflux
  • Environmental factors: metal powders and sawdust inhalation are associated with this disease
  • Infectious agents: a high prevalence of Epstein-Barr virus, CPV, HIV and Hepatitis C has been found in patients suffering from pulmonary fibrosis

Symptoms can vary from no symptoms whatsoever to experiencing fatigue after any form of activity.

The most common symptoms are shortness of breath, a cough which develops over months or years, and, in advanced stages, respiratory failure resulting in a need for oxygen.

In other cases symptoms can include fever, weight loss and clubbing of the fingers or toes.


What are the most common symptoms?

  • Shortness of breath
  • A cough
  • Fatigue
  • Fever

 

Pulmonary fibrosis is a chronic disease that causes the lung lose their flexibility and their function will deteriorate. [Video only available in Spanish]

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