Pulmonary fibrosis

Pulmonary fibrosis is a group of chronic lung diseases caused by progressive scarring of the lung tissue. It also impacts the connecting tissue in your lungs and the air sacs (alveoli) inside them.

As the scar tissue thickens and stiffens, your lungs can’t expand as well as they should, making it harder for you to breathe. You may tire out much easier, even during everyday tasks.

While there is no cure, we offer various treatments that can slow disease progression and keep your symptoms under control.

There are many types of pulmonary fibrosis, including:

• Asbestosis (an asbestos-related lung disease)
• Drug-induced pulmonary fibrosis
• Hypersensitivity pneumonitis
• Idiopathic pulmonary fibrosis
• Idiopathic non-specific interstitial pneumonia
• Pneumoconiosis
• Rheumatoid arthritis interstitial lung disease
• Sarcoidosis
• Silicosis
• Systemic sclerosis

The type of pulmonary fibrosis you have can help us determine the right treatment options for you.

Pulmonary fibrosis can cause different symptoms in different people. However, common pulmonary fibrosis symptoms include:

• Shortness of breath
• A persistent, dry cough
• Fatigue
• Unexplained weight loss
• Breathing in short, shallow breaths
• Chest discomfort

As pulmonary fibrosis progresses and becomes more severe, some people develop clubbing (swelling) of their fingertips and toes and cyanosis (where your skin is tinged blue or grey).

To diagnose pulmonary fibrosis, we’ll first ask about your symptoms, medical history and potential exposure to harmful substances. Then, we’ll perform a physical exam, listening to your chest and checking for abnormalities, such as crackling sounds when you breathe.

Diagnostic imaging tests are usually the next step, as they can reveal lung scarring in more detail.

Imaging tests we may perform are:

• Chest X-ray
• Computerised tomography (CT) scan

If imaging tests have not shown any scarring, we may also collect a lung biopsy (tissue sample) to confirm the condition.

Lung function tests play an important role in diagnosing pulmonary fibrosis because they measure your lung capacity and oxygen exchange capacity to see how much pulmonary fibrosis is affecting your lungs. We’ll also check your oxygen levels to see how well your lungs work.

Sometimes, we may perform blood tests to check for underlying autoimmune diseases that may have led to pulmonary fibrosis.

Various factors can cause pulmonary fibrosis.

Some of the potential causes of pulmonary fibrosis are:

• Inhalation of harmful substances like asbestos, coal dust and silica dust – this is typically more common in people working in environments that expose them to dangerous materials
• Autoimmune diseases like rheumatoid arthritis and lupus can cause inflammation and scarring in your lungs
• Radiation exposure during cancer treatment can lead to scarring of the lungs

Other factors can affect your risk of developing pulmonary fibrosis. For example, smoking can lead to long-term lung damage and pulmonary fibrosis.

However, in some cases, the cause of pulmonary fibrosis can remain unknown.