Respiratory Medicine

Pulmonary Fibrosis

Introduction to Pulmonary Fibrosis

Pulmonary fibrosis is a disease affecting the tiny air sacs of the lung called the alveoli. It is a rare condition, affecting approximately 2 in 10,000 people.

Written by Doctify Team 27/04/2020

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease affecting the tiny air sacs of the lung called the alveoli. It is a rare condition, affecting approximately 2 in 10,000 people. The number of people with this disease is increasing. It is more common in the older age group, however can affect anybody at any age.

What are the causes for Pulmonary Fibrosis?

Pulmonary fibrosis can also be known as idiopathic pulmonary fibrosis. Idiopathic means unknown cause. Although the exact cause is unknown the current theory is that the cells that line the alveoli are damaged in some way. Theses cells then try to heal themselves, creating thickening to the walls of the alveoli. This thickening reduces the amount of oxygen that can get into the blood.

It is thought that there is an association between pulmonary fibrosis and various factors, including: cigarette smoking, viral infections, certain medications, pollutants, gastro-oesophageal disease and family history of pulmonary fibrosis.

What are the symptoms of Pulmonary Fibrosis?

Symptoms tend to develop gradually. However you can get acute exacerbation of the disease where your symptoms can quickly become a lot worst. The main symptoms of pulmonary fibrosis include: Worsening shortness of breath (this is caused by the reduction is oxygen getting from your lungs into the blood), a dry cough, finger clubbing (this is when the base of your nail starts to swell), tiredness or regular chest infections.

How is Pulmonary Fibrosis treated?

To confirm the diagnosis of pulmonary fibrosis, a CT scan and a lung biopsy may be required.

There is currently no cure for pulmonary fibrosis. Therefore the aim of treatment is to reduce the severity of symptoms as best as possible.
Steroid medication is usually effective in approximately 25% of cases. A high dose of steroids is used initially and then tapered down to a maintenance dose.
Oxygen treatment can also be used if symptoms become severe.
Pulmonary rehabilitation courses can provide education about the disease as well as the benefits of physical exercise.
You should stop smoking and get immunisations against pathogens that are likely to give you a chest infection.
A lung transplant may be an option in younger individuals who develop severe disease.

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