Pulmonary fibrosis symptoms

Pulmonary fibrosis symptoms

Symptoms of pulmonary fibrosis often develop gradually and worsen over time. The severity varies; some may experience severe breathlessness, while others may not show significant symptoms for years. The most common symptoms include the following:

• Shortness of breath, or breathlessness

• Persistent dry cough

• Extreme tiredness

• Loss of appetite and weight loss

• Clubbed fingertips (rounded and swollen)

Each of these symptoms progressively impacts daily living, and early detection is important.

At first, the symptoms might appear trivial and be put down to ‘aging’ – slight breathlessness when climbing stairs or a persistent dry cough. A closer look at each symptom provides a clearer understanding of what to look out for.

Shortness of breath

Breathlessness is one of the primary symptoms of pulmonary fibrosis. The condition scars and stiffens the tiny air sacs in the lungs, hampering oxygen exchange. Initially, shortness of breath may occur only during physical exertion, like walking or getting dressed, but as the disease advances, even minimal exertion can cause breathlessness, significantly impacting quality of life. Over time, the lungs work harder and harder to provide the body with enough oxygen, leading to extreme tiredness and a noticeable decline in lung function.

Persistent dry cough

Another common symptom of pulmonary fibrosis is a persistent dry cough. Unlike other coughs, this one is typically non-productive, meaning it does not produce mucus. This persistent cough can be debilitating, often interfering with sleep and daily activities.

Tiredness and weight loss

Tiredness and unintended weight loss are also common symptoms of pulmonary fibrosis. Extreme tiredness, or fatigue, can significantly impact the ability to perform everyday tasks and enjoy life. Unintentional weight loss can occur gradually, further weakening the body and overall health. Unfortunately, some of the medications for lung fibrosis, such as Ofev, can cause further loss of appetite and weight loss.

Clubbed nails

Clubbing of the fingernails or toenails, also known as digital clubbing, is a common symptom in pulmonary fibrosis and other chronic lung diseases. This involves changes in the shape and appearance of the nails and fingertips, often characterised by:

1. Swollen fingertips: The tips of the fingers or toes become rounded and enlarged.

2. Curved nails: The nails curve more than normal, giving a “clubbing” appearance.

3. Loss of nail bed angle: The angle between the nail and the nail bed flattens or disappears.

Why does clubbing occur in pulmonary fibrosis? No one knows what causes clubbing but one theory is that abnormal blood vessels develop that allow blood to pass through the lungs without going via the smallest blood vessels (shunting). This then allows growth factors that would normally get trapped in the lungs to get trapped in the small blood vessels of the fingers and toes promoting the growth of connective tissue.

Early warning signs

Mild breathlessness or subtle changes in energy levels may signal early pulmonary fibrosis. These early signs are often dismissed as part of ‘aging’ or other less serious conditions. However, even light activities like getting dressed can lead to shortness of breath in individuals with idiopathic pulmonary fibrosis. This mild breathlessness can indicate that the body’s oxygen supply is insufficient.

Fast and shallow breathing is another early warning sign, occurring as the lungs struggle to meet oxygen demands. Additionally, clubbing, characterised by the widening and rounding of the fingers or toes, may indicate the presence of pulmonary fibrosis. Identifying these early signs is crucial for timely intervention and disease management.

Advanced symptoms

As pulmonary fibrosis progresses, symptoms become more severe and complications can arise. One such complication is pulmonary hypertension, which means increased blood pressure in the lungs, a condition that significantly increases morbidity and mortality rates. This condition can lead to a significant decrease in exercise capacity, making everyday activities increasingly difficult.

Another severe complication is right-sided heart failure, which occurs due to the increased pressure in the pulmonary arteries when there is pulmonary hypertension. Patients with advanced pulmonary fibrosis and right heart failure may show physical signs, such as swelling of the ankles.

Causes and risk factors

Pulmonary fibrosis can develop from various causes and risk factors. Idiopathic pulmonary fibrosis (IPF) accounts for a significant number of cases. It is called idiopathic pulmonary fibrosis as the cause of the disease is unknown. Other triggers include environmental and occupational exposures, such as inhaling hazardous materials like asbestos and silica. Quitting smoking is crucial for individuals at risk, as smoking can exacerbate lung damage.

Knowing the causes and risk factors helps in prevention and early detection. Three main categories to consider are environmental and occupational factors, genetic predisposition and idiopathic pulmonary fibrosis (IPF).

Environmental and occupational factors

Environmental factors significantly contribute to developing pulmonary fibrosis. Monitoring air quality and avoiding pollution can help prevent exacerbations in lung conditions.

Looking after your lungs in the more extreme seasons is also important. Read our tips on summer health and winter wellness.

Avoiding lung irritants like dust and chemicals is also crucial for preventing further damage lung tissue and maintaining respiratory health. Read about the health risks causes by silica dust.

Genetic predisposition

A small portion of individuals with idiopathic pulmonary fibrosis have other family members with the disease, suggesting a genetic component. In most cases, IPF appears sporadically, affecting typically one individual in a family.

Read our page on pulmonary fibrosis and genetics.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) lacks an identifiable cause. This interstitial lung disease typically manifests symptoms in individuals between 50 and 70 years of age. The condition makes the lungs stiff due to damaged and scarred alveoli, making oxygen transfer difficult. It is rare for individuals under 50 to develop IPF, and it affects approximately 3-5 million worldwide.

There is currently no cure for IPF, and managing gastroesophageal reflux disease (GERD), which is common in these patients, may help alleviate some symptoms. Medications for managing GERD may be recommended, as it can exacerbate IPF.

Diagnosis of pulmonary fibrosis

Diagnosing pulmonary fibrosis requires a review of a patient’s medical and family history, along with a physical examination. The doctor may inquire about factors such as work, smoking habits, medication, other symptoms of autoimmune rheumatic disease and any other exposures that might affect the lungs. The doctor discusses symptoms and examines the patient to assess health and identify signs of pulmonary fibrosis.

Various tests confirm the diagnosis, including pulmonary function tests, imaging tests and blood tests. Each test provides crucial information about lung function and the extent of lung damage.

Pulmonary function tests

Pulmonary function tests, often known as lung function tests, assess how well the lungs move air, evaluating their ability to breathe air in and breathe out. Spirometry is one of the simplest and most common tests for checking lung function. Patients usually also undergo measurement of transfer factor which looks at how well each unit of the lung works at transferring oxygen from the lungs into the bloodstream.

Imaging tests

Imaging tests like chest X-rays and high-resolution CT scans let the doctor see the extent of lung tissue damage and can identify specific fibrosis patterns. These play a significant role in diagnosing and managing pulmonary fibrosis.

Blood tests

Blood tests are essential for diagnosing pulmonary fibrosis. Arterial blood gas testing measures the levels of carbon dioxide and oxygen in the blood and assesses gas exchange efficiency. Blood tests can also detect possible infections that may complicate the condition.

Treatment options

Pulmonary fibrosis has no cure other than lung transplantation, so treatment primarily focuses on managing symptoms and slowing disease progression. Palliative care supports those with severe cases, and can help with comfort and quality of life. Treatments are tailored to the specific type of pulmonary fibrosis, with newer medications like nintedanib and pirfenidone showing promise in slowing lung scarring.

Medications such as antifibrotic agents help reduce lung damage, while corticosteroids or opioid-based treatments like codeine and morphine can alleviate inflammation or breathlessness. Pulmonary rehabilitation combines structured exercise and education to improve lung function and overall wellbeing.

Preventive measures focus on reducing risk factors like exposure to environmental pollutants and smoking. Maintaining a healthy lifestyle through regular exercise, a balanced diet, and protective measures in hazardous environments can significantly lower the risk of developing pulmonary fibrosis. Quitting smoking and avoiding irritants are also essential steps to preserve lung health.

Complications of pulmonary fibrosis

Patients with pulmonary fibrosis may develop pulmonary hypertension, potentially worsening progression to respiratory failure. Arrhythmias, such as atrial fibrillation, are more common in pulmonary fibrosis patients. They are at risk of developing life-threatening lung infections, especially if they are on immunosuppressive therapy. Palliative care focuses on managing symptoms and improving the quality of life for patients with advanced illness.

Living with pulmonary fibrosis

Living with pulmonary fibrosis requires patients to take an active role in their own care and being open and honest with their doctors and healthcare team. Patients should express their concerns and questions to ensure they receive the best possible care, which includes end of life care. Emotional support from family and friends is important for those adjusting their lifestyle to cope with pulmonary fibrosis.

Check out support groups from Asthma and Lung UK and Action for Pulmonary Fibrosis.

Using relaxation techniques and breathing exercises can help reduce anxiety caused by breathlessness and improve overall ability to cope with the condition. These strategies can also contribute to better disease management and quality of life.