What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a health condition involving the progressive scarring and thickening of the lungs. This occurs without a known cause and can lead to the presentation of symptoms such as cough, shortness of breath, hypoxia and fatigue.

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Epidemiology of idiopathic pulmonary fibrosis

On a global basis, there are estimated to be 10.7 cases of idiopathic pulmonary fibrosis per 100,000 persons for males and 7.4 cases for females. The prevalence of the condition is approximately 20 cases per 100,000 population for males and 13 cases for females.

There is a poor prognosis for patients with idiopathic pulmonary fibrosis and the average survival following diagnosis is estimated to be between two and five years. The mortality rates range from 58 deaths per million people for women and 64 for men.

Pathology of idiopathic pulmonary fibrosis

Although the cause of idiopathic pulmonary fibrosis is unknown, there are a number of factors that can contribute to the pathology and increase the probability that an individual will be affected by the condition. These include a history of smoking, an inherited genetic susceptibility and exposure to triggers in the environment, such as certain dust particles or fumes.

The normal structure and function of the lungs can change, slowly progressing with more damage. Initially, scar tissue forms on the alveoli in the lungs, which gradually gets thicker. Over time, this can interfere with the air passing through the lungs and can make it difficult to breathe and supply the oxygen needed in the blood.

Most individuals that present with idiopathic pulmonary fibrosis are over the age of 60, and it is rare for the condition to be diagnosed before the age of 40. This is likely associated with the pathophysiology of the condition, especially the fact that continuous damage over time appears to be the prime risk factor.

Symptoms of idiopathic pulmonary fibrosis

Most patients that are diagnosed with idiopathic pulmonary fibrosis are over the age of sixty, and anyone over the age of 40 is considered to be at risk of the condition.

  • Shortness of breath
  • Dry cough
  • Hypoxia of the blood
  • Fatigue

It is common for individuals to have difficulty partaking in daily activities as usual due to difficulty breathing. For example, they may feel the need to slow down or rest during simple physical activities, such as when walking or climbing stairs.

Diagnosis of idiopathic pulmonary fibrosis

The diagnosis of idiopathic pulmonary fibrosis involves questioning about the presenting symptoms and medical history, in addition to a physical examination and further tests if needed.

It is important to obtain a complete picture of the individual’s circumstances, including other health conditions that may be involved and unique risk factors such as the occupational environment.

Lung function tests are then conducted to assess the ability of the lungs to perform their role and provide oxygen to the body via the blood. Imaging tests such as X-ray and computed tomography (CT) scan can also provide valuable information about the changes to the lung tissue that may be indicative of IPF. In some cases, a lung biopsy can be taken to investigate the cause further.

Idiopathic pulmonary fibrosis - an Osmosis preview

Treatment of idiopathic pulmonary fibrosis

There is no cure for IPF, so the treatment is primarily to relieve symptoms and depends greatly on the circumstances of the individual patient.

Oxygen support and pulmonary rehabilitation are two techniques that can be used to help relieve shortness of breath and improve the quality of life.

Pharmacological treatment is sometimes useful to improve symptoms and slow down the progression of the disease. This may include treatment with pirfenidone or N-acetylcysteine, depending on the patient. There are also other drugs in development for this indication, such as Nintedanib.

Some patients may benefit from a lung transplant to improve the function of the lung, although this is a significant procedure with that carries the risk of transplant rejection and infection.


Further Reading

Last Updated: Apr 13, 2021

Yolanda Smith

Written by

Yolanda Smith

Yolanda graduated with a Bachelor of Pharmacy at the University of South Australia and has experience working in both Australia and Italy. She is passionate about how medicine, diet and lifestyle affect our health and enjoys helping people understand this. In her spare time she loves to explore the world and learn about new cultures and languages.


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