Frequently asked questions about Pulmonary Fibrosis

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What is pulmonary fibrosis?

It is a process in which the lung tissue is damaged and produces scars that gradually replace the healthy tissue, which leads to a gradual worsening of symptoms and lung function. 

What does it mean if a pulmonary fibrosis is idiopathic?

It means that a specific cause has not been identified. Idiopathic pulmonary fibrosis is the most known fibrosing interstitial lung disease. 

Pulmonary fibrosis is a rare disease, but how many people does it affect in Spain?

In Spain, per 100,000 inhabitants 7.6 cases of diffuse interstitial lung disease (DILD) are diagnosed. The most frequent of the idiopathic DILD is IPF, and it is estimated that there are currently between 7,500 and 10,000 patients in Spain with this disease. 

What are the main risk factors of pulmonary fibrosis?

The main risk factors are: family history or genetic predisposition; smoking; age and gender (more common in older men); type of work, hobbies, home environment; previous treatments: chemotherapy, radiotherapy, and autoimmune diseases. 

What is the main symptom of Pulmonary Fibrosis?

Dyspnoea or feeling of breathlessness on exertion. 

What does it mean when the doctor mentions that Velcro-type crackles are heard in the lung?

The Velcro-type dry crackles are characteristic sounds of fibrosing interstitial lung diseases (ILD) and, in particular, pulmonary fibrosis. Its name arises from the noise made by Velcro on separating its two layers, which similar to what the doctor hears in the auscultation with the stethoscope. 

What is acropachy?

It is the term used to refer to “club fingers”. The characteristic of this sign is the painless and insensitive enlargement of the ends of the fingers and toes, due to a thickening of the tissue that is found below the nails of the fingers and toes. The nail is curved downwards, similar to the shape of the rounded part of a spoon turned upside down. It is a common sign in cases of idiopathic lung fibrosis, although it can be present in another type of disease. 

How are fibrosing diffuse interstitial lung diseases diagnosed?

For a proper diagnosis of these very complex diseases a multidisciplinary team is required, which includes different specialists such as chest physicians, radiologists, pathologists, and specialist in autoimmune diseases, among others, who have experience in the management of interstitial lung diseases.

What tests are necessary for the diagnosis of diseases that involve pulmonary fibrosis?

The following diagnostic tests are generally required: laboratory tests; lung imaging tests: chest X-ray and chest CT scan; respiratory function tests and walking test; fibre bronchoscopy and / or lung biopsy using video thorascopy.  

When is it necessary to perform a surgical lung biopsy using video thoracoscopy?

In those cases where the diagnostic tests are performed, and after a multidisciplinary assessment of the risks and benefits, it is considered that it is necessary in order to reach a diagnosis of the type of fibrosing diffuse interstitial lung disease. 

What are the main components of the non-pharmacological treatment of fibrosing DILDs?

Home oxygen therapy in the cases indicated, education on healthy lifestyles, lung rehabilitation, and vaccination for the prevention of respiratory infections. 

What is the main pharmacological treatment of pulmonary fibrosis?

Anti-fibrosis treatments: pirfenidone and nintedanib.  

What are the most common side effects of the pharmacological treatment with nintedanib?

Diarrhoea (62%), nausea (24%), vomiting (12%), and changes in liver function (14%). 

What are the most common side effects of the pharmacological treatment with pirfenidone?

Skin rash (30%), photosensitivity (9%), nausea (36%), abdominal discomfort (24%), dyspepsia (19%), anorexia (13%), fatigue (26%), and elevation of transaminase levels more than three times the normal value (4%). 

What is the mean survival of idiopathic pulmonary fibrosis if no medical treatment is received?

After the initial diagnosis of the disease, it has a mean survival that varies between two and five years. 

What are the most common complications of pulmonary fibrosis?

The complications are separated into: acute (exacerbations of the fibrosing DILD), and chronic (pulmonary hypertension, gastro-oesophageal reflux, SAHS, lung cancer, pneumothorax).  

How can the gastrointestinal side effects of the anti-fibrosis drugs be prevented?

For the prevention of the gastrointestinal effects it is recommended to take the drug during meals; to eat small amounts of food more often throughout the day instead of a few large meals; and to avoid foods that are fried, rich in fats, or spicy, as well as fizzy drinks. If there is nausea or a change in gastrointestinal rhythm, prokinetic drugs (that help the intestinal transit) can be prescribed, as well as a gastric protector, antidiarrhoeals to minimise the symptoms, and to provide dietary recommendations arising from the consultation with the nutritionist. If the adverse effects are more common at a specific time of the day (morning, afternoon, or night), the doses could be reduced. If this measure is also ineffective, the withdrawal of the drug for 10-15 days will be assessed, being able to be re-introduced gradually after the symptoms improve.  

How can the skin side effects of the anti-fibrosis drugs, pirfenidone in particular, be prevented?

As regards the skin effects (photosensitivity, skin rash), it is advised to avoid exposure to the sun in hours of maximum intensity (midday or first hours of the afternoon); the use of hats, sunglasses, long trousers and long-sleeve T-shirts, as well as a sun protector with a protection factor greater than 50 against UV-A and UV-B rays. In cases where a skin rash persists for more than 7 days, the drug should be withdrawn for 15 days and re-introduced gradually after the rash is resolved. If the skin rash has urticaria characteristics or develops in areas of the body not exposed to sunlight, it must be considered of allergic origin. In this case, the drug should be withdrawn permanently and treatment with antihistamines and oral prednisone should be prescribed.  

Can someone travel in an aeroplane if they have pulmonary fibrosis?

Prior to flying, it would be advisable to request medical advice, performing arterial blood gases in indicated cases and/or a hypoxaemia test that could determine the need for supplementary oxygen therapy during the flight.  

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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