Understanding Idiopathic pulmonary fibrosis: Causes, Symptoms, and Management

What is it?

Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.
It's not clear what causes it, but it usually affects people who are around 70 to 75 years old, and is rare in people under 50.
Several treatments can help reduce the rate at which IPF gets worse, but there's currently no treatment that can stop or reverse the scarring of the lungs.

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Additional names

This group contains additional names:
- IPF

Signs & symptoms

The symptoms of IPF tend to develop gradually and get slowly worse over time. Symptoms can include:
* shortness of breath
* a persistent dry cough
* tiredness
* loss of appetite and weight loss
* rounded and swollen fingertips (clubbed fingers)

Many people ignore their breathlessness at first and blame it on getting old or being out of shape.
But eventually even light activity such as getting dressed can cause shortness of breath.

Diagnosis

A diagnosis of IPF requires:
* Exclusion of known causes of ILD, e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity
* The presence of a typical radiological pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT).

In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy.

Treatment

There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression. Treatments include:
* self care measures, such as stopping smoking, eating healthily and exercising regularly
* medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib
* breathing oxygen through a mask – you can do this while you're at home or while you're out and about
* exercises and advice to help you breathe more easily (pulmonary rehabilitation)
* a lung transplant – this is suitable in a few cases, although donor lungs are rare

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

Learn more about our editorial process for content accuracy.

Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:

National Institution of Health ∙ World Health Organization ∙ University of Illinois hospital