Pulmonary Fibrosis: Symptoms, Causes & Treatment Options

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a chronic, progressive lung disease characterized by scarring (fibrosis) of the lung tissue. This scarring thickens and stiffens the delicate lung tissue, making it increasingly difficult for the lungs to function properly and transfer oxygen to the bloodstream. The condition is classified as a restrictive lung disease because the scarring restricts the lungs' ability to expand fully.

The lungs contain millions of tiny air sacs called alveoli, where oxygen from the air we breathe is transferred to our blood. In pulmonary fibrosis, the tissue around and between these air sacs becomes damaged and scarred. This scarring is called fibrosis, from the Latin word "fibra" meaning fiber. As more scar tissue develops, the lungs become progressively stiffer and less able to expand during breathing.

Unlike healthy lung tissue, which is soft and elastic, fibrotic lung tissue is thick and rigid. This rigidity means that people with pulmonary fibrosis must work harder to breathe, even during simple activities that previously required no extra effort. The scarred tissue also impairs the transfer of oxygen from the lungs into the bloodstream, leading to low blood oxygen levels (hypoxemia), which causes fatigue and shortness of breath.

Pulmonary fibrosis is a relatively uncommon condition, affecting approximately 13 to 20 people per 100,000 worldwide. However, its incidence is increasing, partly due to better diagnostic techniques and an aging population. The disease primarily affects adults over 50 years of age, with the risk increasing significantly with advancing age. Men are affected slightly more often than women.

The disease can progress at different rates. Some people experience a slow decline in lung function over many years, while others may deteriorate more rapidly. In some cases, patients may experience acute exacerbations—sudden, severe worsening of symptoms that can be life-threatening. Understanding the nature of this disease and seeking early medical attention are crucial for managing symptoms and maintaining quality of life.

Types of Pulmonary Fibrosis

Pulmonary fibrosis encompasses several distinct conditions, each with different causes and characteristics. The most common and well-known type is idiopathic pulmonary fibrosis (IPF), where "idiopathic" means the cause is unknown. IPF typically affects older adults and tends to progress steadily over time. It represents roughly 20-30% of all interstitial lung disease cases.

Other forms of pulmonary fibrosis are associated with identifiable causes or underlying conditions. These include fibrosis caused by occupational exposures (such as asbestosis or silicosis), fibrosis related to autoimmune diseases (such as rheumatoid arthritis or systemic sclerosis), drug-induced fibrosis, and radiation-induced fibrosis. When the cause can be identified, addressing the underlying factor may help slow or halt progression.

What Are the Symptoms of Pulmonary Fibrosis?

The main symptoms of pulmonary fibrosis include progressive shortness of breath (especially during physical activity), a persistent dry cough, fatigue and reduced energy, unexplained weight loss, and finger clubbing. Symptoms typically develop gradually over months to years, which often delays diagnosis.

Pulmonary fibrosis symptoms usually develop slowly and may initially be attributed to aging, being out of shape, or other more common conditions. This gradual onset often means that significant lung damage has already occurred by the time a diagnosis is made. Recognizing the early signs and seeking medical evaluation promptly is essential for starting treatment as early as possible.

The hallmark symptom of pulmonary fibrosis is progressive dyspnea—shortness of breath that worsens over time. Initially, breathlessness may only occur during strenuous activities such as climbing stairs, exercising, or walking uphill. As the disease progresses, even routine activities like getting dressed, cooking, or walking across a room can become exhausting. In advanced stages, shortness of breath may occur even at rest.

A persistent dry, hacking cough is another common symptom. Unlike a productive cough that brings up phlegm, the cough associated with pulmonary fibrosis is typically dry and can be quite distressing. It may worsen over time and can significantly impact quality of life, interfering with sleep and daily activities.

Complete List of Symptoms

• Progressive shortness of breath (dyspnea): Worsens over time, initially during activity, later at rest
• Dry, persistent cough: Usually non-productive, may be worse at night
• Fatigue and weakness: Feeling tired even after rest, reduced energy for daily activities
• Unexplained weight loss: The body uses more energy for breathing, and eating may become difficult
• Finger clubbing: Widening and rounding of the fingertips and nails
• Aching muscles and joints: General discomfort throughout the body
• Chest discomfort: Tightness or mild pain in the chest
• Loss of appetite: Reduced desire to eat, possibly due to fatigue or breathing difficulty

Periods When Symptoms Worsen

People with pulmonary fibrosis may experience periods when their symptoms suddenly become worse. These are called acute exacerbations and can be triggered by respiratory infections, aspiration of stomach contents, environmental irritants, or sometimes occur without any identifiable cause. During an exacerbation, breathlessness may dramatically increase over days to weeks, and patients may require hospitalization.

Respiratory infections pose a particular risk for people with pulmonary fibrosis. Common colds and other viral infections that would cause only mild symptoms in healthy individuals can lead to serious complications and prolonged illness in those with compromised lung function. This is why prevention through vaccination and avoiding sick contacts is so important.

When Should You See a Doctor for Breathing Problems?

See a doctor if you experience persistent shortness of breath, especially if it's getting worse over time, or if you have a cough lasting more than 8 weeks. Seek immediate medical attention if you have sudden severe breathing difficulty, chest pain, or if you cough up blood. If you have known pulmonary fibrosis, contact your healthcare team if symptoms worsen despite treatment.

Many people delay seeking medical attention for breathing problems because symptoms develop gradually and are often attributed to aging, being overweight, or being out of shape. However, early evaluation is crucial because pulmonary fibrosis and other serious lung conditions are most effectively managed when caught early. Don't dismiss persistent breathlessness as a normal part of getting older.

Schedule an appointment with your doctor if you experience any of the following symptoms that persist for more than a few weeks:

• Shortness of breath during activities that previously didn't cause you to feel winded
• A dry cough that doesn't go away after 8 weeks
• Increasing fatigue that isn't explained by other factors
• Unexplained weight loss
• Changes in the appearance of your fingernails or fingertips

If You Already Have Pulmonary Fibrosis

If you've been diagnosed with pulmonary fibrosis, it's important to stay in regular contact with your healthcare team and report any changes in your condition promptly. Contact your doctor if you notice your breathlessness is getting worse, your cough increases or changes character, you're requiring more supplemental oxygen than usual, or you develop signs of infection such as fever, increased sputum, or worsening fatigue.

What Causes Pulmonary Fibrosis?

Pulmonary fibrosis can result from various causes including occupational exposures (asbestos, silica, coal dust), certain medications, radiation therapy, and autoimmune conditions like rheumatoid arthritis. When no cause can be identified, it's called idiopathic pulmonary fibrosis (IPF). Smoking, older age, and genetic factors also increase the risk of developing this condition.

Understanding what causes pulmonary fibrosis is important for both prevention and treatment. While in many cases the exact cause cannot be determined, identifying contributing factors can help guide treatment decisions and potentially slow disease progression. The lungs appear to become scarred as a result of repeated injury to the lung tissue, followed by an abnormal healing response that produces excessive scar tissue instead of healthy tissue.

The various causes of pulmonary fibrosis can be broadly categorized into occupational and environmental exposures, medical treatments and medications, autoimmune conditions, and unknown causes (idiopathic). Each category includes specific risk factors that healthcare providers consider when evaluating patients with suspected pulmonary fibrosis.

Occupational and Environmental Exposures

Long-term exposure to certain dusts and particles in the workplace is a well-established cause of pulmonary fibrosis. Workers in certain industries face higher risks due to chronic inhalation of harmful substances. The lungs' constant exposure to these particles triggers ongoing inflammation and eventually leads to scarring. Common occupational causes include:

• Asbestos: Used historically in construction, insulation, and shipbuilding. Even exposure decades ago can lead to asbestosis.
• Silica dust: Found in mining, sandblasting, construction, and glass manufacturing
• Coal dust: Affects coal miners (coal workers' pneumoconiosis or "black lung")
• Metal dusts: Including hard metal dust containing tungsten carbide
• Moldy hay and grain: Can cause farmer's lung (hypersensitivity pneumonitis)
• Bird droppings: Can cause bird fancier's lung
• Chemical fumes: Various industrial chemicals can damage lung tissue

Tobacco Smoking

Cigarette smoking is a significant risk factor for pulmonary fibrosis. The toxic substances in tobacco smoke cause chronic inflammation and damage to the delicate lung tissue. Current and former smokers have a substantially higher risk of developing idiopathic pulmonary fibrosis compared to never-smokers. Smoking can also accelerate the progression of existing lung fibrosis and reduce the effectiveness of treatments.

The good news is that quitting smoking at any stage can help slow disease progression. If you smoke and have been diagnosed with pulmonary fibrosis—or are at risk for it—quitting should be a top priority. Your healthcare team can provide resources and support to help you quit successfully.

Medical Treatments and Medications

Certain medical treatments can cause pulmonary fibrosis as a side effect. Radiation therapy to the chest, commonly used to treat breast cancer, lung cancer, and lymphoma, can damage lung tissue and lead to radiation-induced fibrosis. The risk depends on the radiation dose, the area treated, and whether chemotherapy is also given.

Various medications have also been linked to pulmonary fibrosis, including some chemotherapy drugs (bleomycin, methotrexate), certain heart medications (amiodarone), some antibiotics (nitrofurantoin), and certain anti-inflammatory drugs. If you're taking any medications long-term, your doctor should monitor for potential lung effects.

Autoimmune and Connective Tissue Diseases

Several autoimmune conditions can cause or contribute to pulmonary fibrosis. In these diseases, the immune system mistakenly attacks the body's own tissues, including the lungs. Conditions associated with pulmonary fibrosis include:

• Rheumatoid arthritis: Up to 10% of patients develop significant lung involvement
• Systemic sclerosis (scleroderma): Lung fibrosis is common and a major cause of mortality
• Sjögren's syndrome: Can affect the lungs in addition to moisture-producing glands
• Dermatomyositis and polymyositis: Inflammatory muscle diseases that can involve the lungs
• Mixed connective tissue disease: Features overlap between several autoimmune conditions

Idiopathic Pulmonary Fibrosis

When no cause can be identified despite thorough investigation, the condition is called idiopathic pulmonary fibrosis (IPF). This is the most common type of pulmonary fibrosis and typically follows a progressive course. While the exact cause remains unknown, research suggests that IPF may result from a combination of genetic susceptibility, environmental factors, and aging-related changes in how the lungs repair themselves.

Genetic factors appear to play a role in IPF. People with family members who have pulmonary fibrosis have a higher risk of developing the condition themselves. Researchers have identified several genes that appear to influence susceptibility to IPF, including genes involved in maintaining the protective lining of the alveoli and in the body's response to cellular stress.

How Is Pulmonary Fibrosis Diagnosed?

Pulmonary fibrosis is diagnosed through a combination of medical history, physical examination, lung function tests (spirometry), and imaging studies. High-resolution CT (HRCT) scanning is the key diagnostic tool, revealing characteristic patterns of fibrosis. In some cases, a lung biopsy may be needed to confirm the diagnosis and identify the specific type of pulmonary fibrosis.

Diagnosing pulmonary fibrosis can be challenging because its symptoms overlap with many other conditions, and the disease may have progressed significantly before symptoms become noticeable. A thorough evaluation typically involves multiple tests and may require consultation with pulmonologists (lung specialists) and sometimes a multidisciplinary team including radiologists and pathologists.

The diagnostic process begins with a detailed medical history, where your doctor will ask about your symptoms, when they started, how they've progressed, and any factors that make them better or worse. Information about your occupation, hobbies, medications, and family history is also important, as these can provide clues about potential causes.

Physical Examination

During the physical examination, your doctor will listen to your lungs with a stethoscope. A characteristic finding in pulmonary fibrosis is a sound called "Velcro crackles" or "inspiratory crackles"—fine crackling sounds heard during breathing that sound similar to Velcro being slowly pulled apart. Your doctor will also look for signs of low blood oxygen, such as bluish discoloration of the lips or fingertips, and will examine your fingers for clubbing.

Lung Function Tests (Spirometry)

Spirometry measures how much air you can breathe in and out and how quickly you can exhale. In pulmonary fibrosis, the pattern typically shows restrictive impairment—reduced lung volumes because the stiff, scarred lungs cannot expand fully. The forced vital capacity (FVC), which measures total lung capacity, is typically reduced. Regular spirometry testing helps track disease progression over time.

Imaging Studies

Chest X-rays may show abnormalities suggestive of pulmonary fibrosis, but the definitive imaging test is high-resolution computed tomography (HRCT). This specialized CT scan provides detailed images of the lung tissue and can reveal characteristic patterns of fibrosis, including:

• Honeycombing: Clustered cystic air spaces with thick walls, resembling a honeycomb
• Reticular pattern: A network of fine lines representing thickened tissue
• Ground-glass opacity: Hazy areas that may indicate inflammation
• Traction bronchiectasis: Distortion of airways pulled by surrounding scar tissue

Additional Tests

Other tests may be performed to complete the diagnostic picture and identify potential causes:

• Blood tests: To check for autoimmune diseases, infection, and general health markers
• Arterial blood gas: Measures oxygen and carbon dioxide levels in the blood
• Six-minute walk test: Assesses functional capacity and oxygen needs during exercise
• Echocardiogram: Evaluates heart function and checks for pulmonary hypertension
• Bronchoscopy: Allows examination of the airways and collection of samples
• Lung biopsy: May be necessary if diagnosis remains uncertain; can be surgical or bronchoscopic

How Is Pulmonary Fibrosis Treated?

Treatment for pulmonary fibrosis aims to slow disease progression, relieve symptoms, and maintain quality of life. Antifibrotic medications (pirfenidone and nintedanib) are the cornerstone of treatment for IPF. Supportive care includes oxygen therapy, pulmonary rehabilitation, and management of complications. For eligible patients with advanced disease, lung transplantation may be considered.

While there is currently no cure for pulmonary fibrosis and existing lung damage cannot be reversed, effective treatments are available that can make a significant difference in how patients feel and function. The goals of treatment are to slow or halt the progression of fibrosis, improve symptoms and quality of life, treat complications, and maintain physical function for as long as possible.

Treatment approaches are individualized based on the type of pulmonary fibrosis, disease severity, patient age and overall health, and patient preferences. A comprehensive treatment plan typically includes medication management, supportive therapies, lifestyle modifications, and regular monitoring to track disease progression and adjust treatment as needed.

Antifibrotic Medications

For idiopathic pulmonary fibrosis, two antifibrotic medications have been shown in clinical trials to slow the decline in lung function:

Pirfenidone (Esbriet) works by reducing the production of growth factors and other substances involved in scar tissue formation. Studies have shown it can slow the rate of lung function decline by approximately 50% compared to placebo. Common side effects include nausea, decreased appetite, and skin rash, particularly sun sensitivity.

Nintedanib (Ofev) is a kinase inhibitor that blocks pathways involved in fibrosis. Like pirfenidone, it has been shown to slow the decline in lung function. The most common side effect is diarrhea, which can usually be managed with dietary modifications and medications.

Both medications can reduce the rate of disease progression, but they don't stop it entirely or reverse existing damage. Starting treatment early, when more healthy lung tissue remains, provides the greatest benefit. Your doctor will help you weigh the benefits and potential side effects of these medications.

Oxygen Therapy

As pulmonary fibrosis progresses, the lungs become less able to transfer oxygen into the blood, leading to low blood oxygen levels (hypoxemia). Supplemental oxygen therapy can help by ensuring adequate oxygen reaches the body's tissues, reducing breathlessness and fatigue, improving ability to exercise and perform daily activities, and protecting the heart from strain.

Oxygen may be prescribed for use during physical activity, during sleep, or continuously, depending on how severe the oxygen deficiency is. Various delivery systems are available, including portable concentrators that allow patients to maintain mobility and independence.

Pulmonary Rehabilitation

Pulmonary rehabilitation is a comprehensive program that combines exercise training, education, and support to help people with lung disease optimize their function and quality of life. Components typically include:

• Supervised exercise: Aerobic and strength training adapted to individual capabilities
• Breathing techniques: Methods to breathe more efficiently and manage breathlessness
• Energy conservation: Strategies to accomplish tasks while minimizing fatigue
• Nutritional counseling: Guidance on maintaining healthy weight and adequate nutrition
• Psychological support: Coping strategies and management of anxiety and depression

Lung Transplantation

For patients with advanced pulmonary fibrosis who meet certain criteria, lung transplantation may be considered. Transplantation can significantly improve survival and quality of life, but it requires lifelong immunosuppressive medication and carries its own risks. Evaluation for transplant candidacy typically considers age, overall health, severity of lung disease, and absence of significant other organ problems.

Because the waiting time for a donor lung can be lengthy, referral for transplant evaluation should occur early enough to allow time for the evaluation process and waiting period. Not everyone with pulmonary fibrosis is a candidate for transplantation, and the decision involves careful discussion between patients, their families, and the medical team.

What Can You Do to Manage Pulmonary Fibrosis?

Self-management strategies for pulmonary fibrosis include staying physically active, using breathing techniques to manage breathlessness, quitting smoking, getting recommended vaccinations, maintaining good nutrition, and addressing emotional health. Following your treatment plan consistently and staying connected with your healthcare team are essential for the best outcomes.

While medical treatment is important, the actions you take at home and in your daily life also play a significant role in managing pulmonary fibrosis. Living well with this condition involves a combination of following medical advice, making healthy lifestyle choices, and developing strategies to cope with symptoms and maintain quality of life.

Many people with pulmonary fibrosis find that they can continue to lead meaningful, active lives by adapting their activities and using the strategies described below. Working with your healthcare team to develop a personalized self-management plan can help you feel more in control of your condition.

Breathing Techniques and Coping with Breathlessness

Learning specific breathing techniques can help you manage breathlessness more effectively. Pursed-lip breathing, where you breathe in slowly through your nose and out through pursed lips, can help slow your breathing rate and make each breath more effective. Diaphragmatic breathing, which emphasizes using your diaphragm rather than chest muscles, can also improve breathing efficiency.

Positioning can also help with breathlessness. Many people find that leaning forward slightly with their arms supported (such as resting on a table or shopping cart) makes breathing easier. Finding positions that work for you during episodes of increased breathlessness can provide relief.

Preventing Respiratory Infections

Respiratory infections can cause significant setbacks for people with pulmonary fibrosis. Protecting yourself from infections is therefore extremely important. Recommended vaccinations include annual influenza (flu) vaccine, COVID-19 vaccines and boosters, pneumococcal vaccines, and any other vaccines your doctor recommends.

Other infection prevention measures include washing hands frequently and thoroughly, avoiding close contact with people who are sick, avoiding crowded places during flu season when possible, and not touching your face with unwashed hands.

Smoking Cessation

If you smoke, quitting is one of the most important things you can do to slow disease progression. Smoking continues to damage lung tissue and accelerates the decline in lung function. It also reduces the effectiveness of treatments and increases the risk of complications. Your healthcare team can provide resources and support to help you quit, including counseling and medications that make quitting easier.

Physical Activity and Exercise

Staying physically active is important even when you have pulmonary fibrosis. Regular activity can improve your endurance and fitness, reduce breathlessness, boost your mood and mental health, maintain muscle strength, and enhance overall quality of life. Work with your healthcare team to develop an exercise plan that's appropriate for your level of lung function. Even gentle activities like walking or chair exercises can be beneficial.

Nutrition and Weight Management

Maintaining good nutrition is important for everyone with pulmonary fibrosis. The extra work of breathing burns additional calories, and some people unintentionally lose weight. Conversely, excess weight can make breathing more difficult. Working with a dietitian can help you develop an eating plan that maintains your energy and supports your overall health.

What Is the Prognosis for Pulmonary Fibrosis?

The prognosis for pulmonary fibrosis varies significantly depending on the type, cause, and severity of the disease. For idiopathic pulmonary fibrosis (IPF), median survival is 3-5 years from diagnosis, though many patients live longer with treatment. Forms of pulmonary fibrosis with identifiable, treatable causes may have better outcomes. Early diagnosis and prompt treatment initiation are associated with better prognosis.

Discussing prognosis for pulmonary fibrosis is challenging because outcomes vary so widely between individuals. Some people experience relatively slow progression and live for many years with a good quality of life, while others may decline more rapidly. The type of pulmonary fibrosis, your overall health, how early the disease is detected, and your response to treatment all influence the outlook.

For idiopathic pulmonary fibrosis specifically, studies have found that the median survival—meaning half of patients live longer and half shorter—is approximately 3 to 5 years from the time of diagnosis. However, it's important to understand that this is an average, and individual trajectories can differ substantially. Some patients have survived 10 years or more after diagnosis, especially with newer treatments and early intervention.

Several factors have been associated with better prognosis in pulmonary fibrosis:

• Younger age at diagnosis
• Better baseline lung function at the time of diagnosis
• Absence of other serious health conditions
• Non-smoking status
• Response to treatment (stable lung function on medication)
• Fibrosis related to treatable underlying conditions

Research into new treatments for pulmonary fibrosis is ongoing and offers hope for improved outcomes in the future. Clinical trials are investigating new medications, combination therapies, and other approaches that may be more effective than current treatments. Ask your doctor about whether any clinical trials might be appropriate for you.