What Is Pulmonary Fibrosis?

Are you having a hard time breathing? Do you get tired easily, even when doing effortless tasks? If so, you might be experiencing the effects of pulmonary fibrosis. 

Pulmonary fibrosis is a condition that stops your lungs from working as they should. As a result, oxygen does not enter your bloodstream as easily and lowers your ability to carry out daily tasks.

Though there is no cure for pulmonary fibrosis, there are options available for you and your doctor to slow its progression and lower its impact on your daily life. 

Overview

Pulmonary fibrosis is a type of interstitial lung disease where fibrous tissue (known as scar tissue) slowly builds up in the lungs, blocking oxygen from entering the bloodstream. This makes breathing difficult, especially when walking and exercising. 

Pulmonary fibrosis can have many causes, such as other diseases, exposure to certain chemicals, and side effects of certain medication. Sometimes, no obvious cause can be identified - this is known as idiopathic pulmonary fibrosis. 

Despite its rarity, pulmonary fibrosis can have a heavy toll on your health and quality of life. 

Causes of pulmonary fibrosis

Many factors can cause or increase your odds of developing pulmonary fibrosis. These can either be related to a certain disease, exposure to environmental or occupational factors, or as a side-effect of some medications.

From exposure

Some substances can lead to disease and increase your risk of developing pulmonary fibrosis. These include:

• Hypersensitivity pneumonitis from inhaled substances that you are allergic to. Some occupations, such as pigeon breeding and farming, are often behind cases of hypersensitivity pneumonitis
• Pneumoconiosis from breathing excessive dust at work. It is given different names depending on the substance. Asbestosis (caused by asbestos) and silicosis (caused by silica dust) are the most common. Other types include black lung disease, affecting predominantly coal workers, and brown lung disease (also known as byssinosis), which is most common in those who work with raw natural textiles

Disease

Some diseases are also known to cause or increase the risk of pulmonary fibrosis, these include:

• Rheumatoid arthritis
• Systemic sclerosis, also known as scleroderma
• Sarcoidosis 

COVID-19, in more severe cases, has also been found to increase the risk of developing pulmonary fibrosis. 

As a side effect of medication

Some medications may increase your risk of pulmonary fibrosis. Some of these include: 

• Chemotherapy, such as bleomycin
• Medication for arrhythmia, such as amiodarone
• Medication for inflammatory conditions, such as methotrexate
• Antibiotics, such as nitrofurantoin

No known cause

Sometimes it is not possible to identify a cause behind your pulmonary fibrosis. This is known as idiopathic pulmonary fibrosis. 

Pulmonary fibrosis runs in some families. If this is your case, you may be diagnosed with familial idiopathic pulmonary fibrosis. 

A specific case of idiopathic pulmonary fibrosis that affects younger people is known as non-specific interstitial pneumonia. 

Signs and symptoms of pulmonary fibrosis

The most obvious sign of pulmonary fibrosis is shortness of breath, especially if you are exercising. Breathlessness may be accompanied by: 

• Fast, shallow breathing
• Dry cough
• Tiredness
• Non-intentional weight loss
• Pain in joints and muscles
• Wide, round finger or toe tips (known as clubbing)

People most commonly begin experiencing symptoms between the ages of 50 and 70.

Make sure to check with your doctor if you feel any of these symptoms. You may have to undergo some tests to identify the cause behind them. 

Management and treatment for pulmonary fibrosis

The objective of treatment is slowing the progression of the disease and minimising its impact on your quality of life by addressing its symptoms. This includes: 

• Breathlessness management
• Pulmonary rehabilitation
• ‍Oxygen therapy if blood oxygen levels fall below a certain level
• ‍Cough medication
• Mucolytics help you clear sputum from the lungs
• Management of anxiety and panic attacks

Depending on the cause behind your pulmonary fibrosis, you may be also given:

• Medications such as nintedanib and pirfenidone for idiopathic pulmonary fibrosis.
• Medications that tone down your immune system, including methotrexate, rituximab, cyclophosphamide, and steroids such as prednisolone if your pulmonary fibrosis is the result of inflammation of the lungs.

There are new therapies for idiopathic pulmonary fibrosis currently being tested in clinical trials. Please discuss with your doctor if you are interested in participating in a clinical trial.

Diagnosing pulmonary fibrosis

If you suffer from any symptoms and your doctor suspects pulmonary fibrosis, you may be referred to a specialist for further examination. This may include: 

• Health history including any exposures to dangerous substances, diseases, as well as family history with lung disease. This will help identify the cause behind your symptoms
• A physical exam where the doctor listens to your lungs
• Lung function tests such as Pulmonary Function Tests (PFT) to measure your lung function and pulse oximetry and arterial blood gas tests to measure the amount of oxygen in your blood
• Imaging tests such as chest X-rays (if in the early stages), CT scans, and high-resolution CT(HRCT) to check for scarring, as well as an echocardiogram (ECHO) to detect pulmonary hypertension
• Taking tissue samples through a bronchoscopy

Other tests may be carried out to diagnose related diseases such as an esophagram to detect gastroesophageal reflux disease (GERD) or a skin or blood test to detect tuberculosis.

Depending on your symptoms, lung function, exercise capacity, and lung health, your pulmonary fibrosis may be classified as mild, moderate, severe, or very severe.

Prevention and outlook

Prevention

Though there is no fail-proof way to prevent pulmonary fibrosis, you may take steps to decrease the risk - such as by minimising exposure to risk factors. This is even more important if your family has a history of idiopathic lung disease. 

Some medications increase the odds of pulmonary fibrosis. If you are already at risk from your occupation or family history, consult your doctor to explore alternative treatments. 

Following a healthy lifestyle, such as exercising and not smoking can decrease the overall risk of developing pulmonary fibrosis.¹

If your work exposes you to substances known to cause pulmonary fibrosis, make sure to follow the appropriate safety guidelines for your occupation.

Outlook

Pulmonary fibrosis is a progressive condition where scar tissue slowly builds up in the lungs. As a result, symptoms such as breathlessness get worse over time. 

Not all patients experience disease progression in the same way and pulmonary fibrosis may develop more quickly in some people than in others based on age, lifestyle, and overall health.

People diagnosed with pulmonary fibrosis are expected to live an average of three to five years, but please note that your case is unique as some people live either more or fewer years.

In some cases, you may experience a sudden worsening of your condition. This is known as an acute exacerbation. It is sometimes a result of a specific triggering event, which will be treated by your doctor if identified. Depending on the severity, you may need to be hospitalised, possibly including ventilation support. 

FAQs

Is pulmonary fibrosis curable?

Aside from a lung transplant, there is currently no cure for pulmonary fibrosis. 

If you are diagnosed with pulmonary fibrosis, the focus is on palliative care and slowing down disease progression. This involves decreasing the impact of symptoms on your quality of life through a combination of medication and lifestyle changes.

Who is at risk of pulmonary fibrosis?

Many factors can increase the risk of pulmonary fibrosis depending on your occupation, exposure to harmful substances, conditions, and medications. Please consult the “Causes of pulmonary fibrosis” section for further details. 

How common is pulmonary fibrosis?

Idiopathic pulmonary fibrosis is a rare disease. About one to thirteen people out of 100,000 are diagnosed with the condition each year, though this varies considerably across countries.² Still, idiopathic pulmonary fibrosis accounts for one in every five cases of interstitial lung disease.³

People assigned male at birth (AMAB) and people over 75 years old more commonly develop pulmonary fibrosis. It is rare in people below 50.

When should I see a doctor?

If you are having a hard time breathing, have been coughing for more than 3 weeks, or are experiencing symptoms related to pulmonary fibrosis, please see a doctor. This will ensure that you are diagnosed as early as possible and the necessary steps can be taken to slow its progression. 

Summary

Pulmonary fibrosis is a rare condition with multiple causes where the lungs progressively lose their ability to function properly. Management involves alleviating symptoms, and identifying and addressing any underlying causes. 

Being diagnosed with pulmonary fibrosis can be a difficult experience. Please be aware that there are organisations such as the Pulmonary Fibrosis Foundation and the Pulmonary Fibrosis Trust that offer support and resources to those diagnosed with pulmonary fibrosis.