Pulmonary fibrosis: how severe can it be?

Pulmonary fibrosis is the term given to the build-up of scar tissue in the lungs, causing thickening and hardening. It can make breathing difficult, resulting in shortness of breath. Esteemed consultant respiratory physician Dr Tuck-Kay Loke, who sees patients in Tunbridge Wells, shares his expertise on this condition, explaining the causes, signs, stages, and treatments. He also details the severity and the life expectancy of someone with pulmonary fibrosis. 

What is pulmonary fibrosis caused by? 

Pulmonary fibrosis has many causes. For example, radiotherapy for the treatment of cancer can, by way of eradicating cancer cells, result in fibrosis of the surrounding tissue. Other conditions, e.g., tuberculosis or sarcoidosis, causes scarring due to an overactive immune response leading to the production of pro-inflammatory substances in lung tissue. In rare cases, the cause is unknown and is termed idiopathic.

What are the signs of pulmonary fibrosis? 

The signs of pulmonary fibrosis are subtle and require a trained clinician to detect.  Lung crackles are frequently heard over the stethoscope, and in advanced cases, the fingernails may show some increase in curvature (also known as clubbing).

What are the different stages of pulmonary fibrosis? 

It is difficult to classify the stages of pulmonary fibrosis. In some ways, this will depend on the condition causing pulmonary fibrosis. Sarcoidosis typically has 4 stages of fibrosis, as defined by progressive radiographic changes on chest X-ray. However, for conditions such as idiopathic pulmonary fibrosis (IPF), the stages may be more difficult to define.

What is the best treatment for pulmonary fibrosis? 

Treatment is usually directed at the cause if this can be identified early. IPF now has licensed treatment aimed at preventing the progression of lung scarring, but this involves taking tablets on a daily basis. Randomised controlled trials have shown these drugs to be effective at preventing a decline in lung function, and can also improve survival in patients with IPF. In the advanced stages of the disease, oxygen therapy is often required to palliate the symptoms of breathlessness and exertional fatigue. 

How serious is pulmonary fibrosis? 

Pulmonary fibrosis can be a very serious condition if not detected early and treated appropriately. In rare cases, pulmonary fibrosis may result in a shortened life expectancy and for selected individuals, a lung transplant may be the only option to prolong life. However, the course and prognosis of this condition can be extremely variable. Patients are therefore advised to seek specialist advice early.

What is the life expectancy of someone with pulmonary fibrosis? 

This varies from disease to disease, and from individual to individual. In typical cases of IPF (where the cause is unknown), life expectancy is usually 3 to 5 years from diagnosis. Conditions such as radiation-induced pulmonary fibrosis, on the other hand, tend to remain pretty static. Smoking tends to accelerate the progression of all forms of pulmonary fibrosis and should be discouraged. Prevention of common viral infections, with seasonal influenza and COVID vaccines, along with a diet rich in antioxidants have been shown to promote lung health and well-being for patients with this condition.

Dr Tuck-Kay Loke is a revered consultant respiratory physician, practising in Tunbridge Wells, with more than 25 years of experience. If you would like to book a consultation with him, you can do so by visiting his Top Doctor’s profile.