Pulmonary Fibrosis: Symptoms, Causes, Diagnosis, and Treatment Explained
Updated: 2 hours ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: September 2026
Pulmonary fibrosis is a lung disease in which lung tissue becomes scarred, thickened, and stiff, making it harder for oxygen to pass into the bloodstream. It usually strikes middle-aged and older adults, and in many cases no cause is ever found — that form is called idiopathic pulmonary fibrosis (IPF). The damage cannot be repaired and no treatment stops the disease outright, but medicines, oxygen therapy, pulmonary rehabilitation, and in some cases lung transplantation can slow the worsening, ease symptoms, and improve quality of life. Sudden rapid worsening (an acute exacerbation) is life-threatening and needs urgent care.
Quick Answer
What happens: Scar tissue forms around and between the air sacs (alveoli), so less oxygen passes into the blood.
Main symptoms: Shortness of breath, dry cough, extreme tiredness, unintended weight loss, aching muscles and joints, and widened, rounded fingertips (clubbing).
Who it affects: Mostly middle-aged and older adults; some types occur in younger people and rarely in children.
Can it be repaired: No — the lung damage cannot be repaired, but treatment can slow it down and ease symptoms.
When to act fast: If breathlessness suddenly worsens over days or weeks (an acute exacerbation), seek urgent care — it can be life-threatening.
What Is Pulmonary Fibrosis?
Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it harder for the lungs to work properly, and the condition worsens over time.
The scarring happens around and between the tiny air sacs in the lungs, called alveoli. These air sacs are where oxygen passes from the air into the bloodstream. When scar tissue forms between them, less oxygen reaches the blood, which is why breathlessness is the hallmark symptom.
How fast the disease worsens varies greatly from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen slowly, over months or years.
The lung damage caused by pulmonary fibrosis cannot be repaired. However, medicines and therapies can sometimes slow down the rate of fibrosis, ease symptoms, and improve quality of life. For some people, a lung transplant might be an option.
What Is Idiopathic Pulmonary Fibrosis?
The scarring in pulmonary fibrosis can be caused by many things — long-term exposure to toxins, radiation therapy, some medicines, and certain medical conditions. But often, healthcare professionals cannot pinpoint what is causing the problem.
When no cause can be found, the condition is called idiopathic pulmonary fibrosis (IPF).
Idiopathic pulmonary fibrosis usually occurs in middle-aged and older adults. Sometimes pulmonary fibrosis is diagnosed in children and infants, but this is not common.
What Does Pulmonary Fibrosis Feel Like? The Symptoms
Symptom | What It Looks Like |
Shortness of breath | The hallmark symptom, growing worse as fibrosis progresses |
Dry cough | A cough that does not bring up phlegm |
Extreme tiredness | Ongoing fatigue from the extra work of breathing and low oxygen |
Unintended weight loss | Losing weight without trying |
Aching muscles and joints | Ongoing soreness in muscles and joints |
Clubbing | Widening and rounding of the tips of the fingers or toes |
How fast the disease worsens and how severe the symptoms are can vary greatly from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.
When to Seek Emergency Care
In people with pulmonary fibrosis, especially idiopathic pulmonary fibrosis, shortness of breath can suddenly get worse over a few weeks or days. This is called an acute exacerbation, and it can be life-threatening. If symptoms suddenly get worse, contact your healthcare team right away.
The cause of an acute exacerbation may be another condition or an illness, such as a lung infection. But usually the cause is not known.
What Causes Pulmonary Fibrosis?
Pulmonary fibrosis is scarring and thickening of the tissue around and between the air sacs in the lungs. Damage that leads to this scarring may be caused by many different things.
Work and Surroundings
The type of work you do and where you work or live could be the cause, or part of the cause. Having continuous or repeated contact with toxins or pollutants — substances that harm the quality of air, water, or land — can damage your lungs, especially without protective gear.
The substances most often linked to pulmonary fibrosis include silica dust, asbestos fibers, hard metal dusts, wood, coal and grain dusts, mold, and bird and animal droppings.
Radiation Treatments
Some people who receive radiation therapy to the chest, such as for lung or breast cancer, show signs of lung damage months or sometimes years after treatment. How severe the damage is may depend on how much of the lung was exposed, the total amount of radiation given, whether chemotherapy was also used, and whether there is underlying lung disease.
Medicines That Can Damage the Lungs
Medicine Group | Examples |
Chemotherapy | Methotrexate (Trexall, Otrexup), bleomycin, cyclophosphamide (Cytoxan) |
Heart medicines | Amiodarone (Nexterone, Pacerone) |
Some antibiotics | Nitrofurantoin (Macrobid, Macrodantin), ethambutol (Myambutol) |
Anti-inflammatory medicines | Rituximab (Rituxan), sulfasalazine (Azulfidine) |
Medical Conditions That Lead to Lung Scarring
Lung damage can also result from a number of conditions, including dermatomyositis (an inflammatory disease marked by muscle weakness and a skin rash), lupus, mixed connective tissue disease, pneumonia, polymyositis, rheumatoid arthritis, sarcoidosis, and scleroderma.
Idiopathic Pulmonary Fibrosis and GERD
Many substances and conditions can lead to pulmonary fibrosis. Even so, in many people, the cause is never found. Risk factors such as smoking or exposure to air pollution could be related, even if the cause cannot be confirmed.
Many people with idiopathic pulmonary fibrosis also have gastroesophageal reflux disease (GERD), a condition in which acid from the stomach flows back into the esophagus. GERD may be a risk factor for IPF or cause it to worsen faster — but more studies are needed.
What Raises Your Risk?
Pulmonary fibrosis has been found in children and infants, but this is not common. Idiopathic pulmonary fibrosis is much more likely to affect middle-aged and older adults. Other types, such as that caused by connective tissue disease, can occur in younger people.
Risk Factor | Why It Matters |
Smoking | Current or past smokers are at higher risk than never-smokers; people with emphysema are at higher risk too |
Certain types of work | Mining, farming, and construction carry higher risk, especially with repeated contact with lung-damaging pollutants |
Cancer treatments | Chest radiation and certain chemotherapy medicines can raise risk |
Genetics | Some types of pulmonary fibrosis run in families |
Complications of Pulmonary Fibrosis
As the disease progresses, pulmonary fibrosis can lead to serious complications.
Complication | What Happens |
Pulmonary hypertension | High blood pressure in the lungs’ arteries; stiff, thick arteries slow or block blood flow, raising pressure in the lungs and the heart’s right chamber (right ventricle) |
Right-sided heart failure | The heart’s right chamber must pump harder than usual to move blood through partly blocked pulmonary arteries |
Respiratory failure | Often the last stage of long-term lung disease; blood oxygen levels fall dangerously low |
Lung cancer | Long-standing pulmonary fibrosis increases the risk of developing lung cancer |
Other lung problems | As the disease worsens, it may lead to blood clots in the lungs, a collapsed lung, or lung infections |
How Is Pulmonary Fibrosis Diagnosed?
To diagnose pulmonary fibrosis, your healthcare professional reviews your medical and family history and does a physical exam. You will likely be asked about any continuous or repeated contact with dusts, gases, chemicals, or similar substances, especially through work.
During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often produces a crackling sound at the base of the lungs.
Imaging Tests
Imaging tests are usually the first step in confirming the diagnosis and gauging how much damage has occurred.
Test | What It Shows |
Chest X-ray | May show the scar tissue usually present in pulmonary fibrosis; sometimes shows no changes, meaning more tests are needed |
High-resolution CT scan | Helps diagnose pulmonary fibrosis and shows how much lung damage has occurred; some kinds of fibrosis have certain patterns |
Echocardiogram | Uses sound waves to view the heart’s structures and function, and shows the pressure in the arteries of the lungs and the right side of the heart |
Lung Function Tests
Lung function tests (also called pulmonary function tests) find out how well your lungs are working.
Test | What It Measures |
Spirometry | How much air the lungs can hold and how quickly air moves in and out, measured by breathing out forcefully through a tube |
Lung volume test | The amount of air the lungs hold at different times during breathing |
Lung diffusion test | How well the body moves oxygen and carbon dioxide between the lungs and the blood |
Pulse oximetry | Oxygen saturation in the blood, using a small device placed on a finger; often paired with a six-minute walking test |
Exercise stress test | Heart and lung function during activity on a treadmill or stationary bike |
Arterial blood gas test | Oxygen and carbon dioxide levels in a blood sample, usually taken from an artery in the wrist |
Imaging and lung function tests are not only used for diagnosis — they also track your condition over time and show whether treatments are working.
Lung Biopsy
If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed in a biopsy, which is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions.
A surgical biopsy is invasive and has potential complications, but it may be the only way to make the right diagnosis. It can be done as video-assisted thoracoscopic surgery (VATS) — a minimally invasive operation with two or three small cuts between the ribs, a camera, and general anesthesia — or as a thoracotomy, an open surgery through a larger cut.
A bronchoscopy removes very small tissue samples — usually no larger than the head of a pin — using a small, flexible tube passed through the mouth or nose into the lungs. The samples are sometimes too small to make the diagnosis, but this procedure may rule out other conditions.
Blood tests may also check your liver and kidney function and help rule out other conditions.
How Is Pulmonary Fibrosis Treated?
The lung scarring and thickening in pulmonary fibrosis cannot be repaired, and no current treatment has proved effective in stopping the disease from worsening over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens; others help improve quality of life.
Treatment depends on the cause of your pulmonary fibrosis. Your healthcare team evaluates how severe your condition is, and together you decide on the best treatment plan.
Medicines
If you have idiopathic pulmonary fibrosis, your healthcare professional may recommend pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the U.S. Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis, and nintedanib is also approved for other types of pulmonary fibrosis that worsen quickly. These medicines may help slow the worsening and may prevent bouts of sudden symptom flare-ups.
Medicine | Possible Side Effects | Monitoring |
Nintedanib (Ofev) | Diarrhea, nausea | Regular blood tests to check liver function |
Pirfenidone (Esbriet) | Nausea, loss of appetite, skin rash from sunlight | Regular blood tests to check liver function |
New medicines and therapies are being developed and tested in clinical trials but are not yet FDA-approved. Researchers continue to study treatments for pulmonary fibrosis.
Doctors may also recommend anti-acid medicines if you have symptoms of GERD, a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.
Oxygen Therapy
Using extra oxygen — called supplemental oxygen — cannot stop lung damage, but it can make breathing and exercise easier, prevent or lessen complications from low blood oxygen levels, possibly lessen strain on the right side of the heart, and improve sleep and a sense of well-being.
You may use oxygen when you sleep or exercise, though some people need it all the time. Carrying a small tank of oxygen or using a portable oxygen concentrator can help you stay mobile.
Pulmonary Rehabilitation
Pulmonary rehabilitation helps manage symptoms and improves your ability to do daily tasks. Programs focus on physical exercise to increase what you can do, breathing techniques that may improve how well your lungs use oxygen, nutritional counseling, emotional counseling and support, and education about your condition.
When Symptoms Suddenly Worsen
During an acute exacerbation, you may need more supplemental oxygen, and in some cases mechanical ventilation in the hospital, where a tube is guided into the lungs and attached to a machine that helps with breathing. Your healthcare professional may also recommend antibiotics, corticosteroid medicines, or other medicines during a sudden worsening.
Lung Transplant
A lung transplant may be an option for some people with pulmonary fibrosis. It can improve quality of life and allow you to live longer, but it can involve complications such as rejection and infection, and you take medicines for the rest of your life afterward. You and your healthcare team may discuss a transplant if it is thought to be the right option for your condition.
Lifestyle and Home Remedies
Being actively involved in your treatment and staying as healthy as possible are essential to living with pulmonary fibrosis.
Stop smoking. If you have lung disease, it is important to stop smoking. Talk with your healthcare team about quitting options, including smoking cessation programs that use proven techniques. Because secondhand smoke can harm your lungs, avoid being around people who are smoking.
Avoid things that irritate your lungs. Indoor pollutants such as fumes from heating fuel or chemicals can irritate your lungs, as can outdoor pollutants like dust or car exhaust.
Eat well. People with lung disease may lose weight both because eating is uncomfortable and because of the extra energy it takes to breathe. A healthy diet with enough calories is needed. Try eating smaller meals more often during the day, and ask a dietitian for guidance.
Get moving. Regular exercise can help you keep your lung function and manage stress. Aim to include activities such as walking or biking in your daily routine. If mobility becomes limited, look for active movements that do not require walking — tai chi is one example.
Take time to rest. Enough rest helps you keep energy and cope with the stress of your condition. If you have trouble sleeping, talk with your healthcare team.
Get vaccinated. Respiratory infections such as colds and flu can worsen symptoms. Get the pneumonia vaccine, an annual flu shot, and COVID-19 vaccines, and make sure family members are vaccinated too. Try to stay out of crowds when possible.
Follow your treatment plan. Ongoing treatment is usually needed. Take medicines as prescribed, adjust diet and exercise as needed, attend pulmonary rehabilitation sessions, keep all appointments, and contact your care team if symptoms worsen.
Coping and Support
Pulmonary fibrosis is a life-long lung condition that worsens over time, and it can cause fear, depression, and stress. Several approaches can help you cope.
Learning about your condition helps you and your family manage it better. Spending time with family and friends — and letting them know how to help — makes a real difference. Talking openly with your healthcare professional about how you feel matters too; if you are depressed or anxious, your doctor may suggest seeing a mental health professional.
Joining a support group lets you meet other people with pulmonary fibrosis who have had similar symptoms or treatments, and you can learn new ways to cope. As the condition worsens, your care team may also suggest palliative care services, which provide support for severe symptoms such as pain relief and help you and your family discuss end-of-life issues and plan advance directives.
Preparing for Your Appointment
If your primary care doctor suspects a serious lung problem, you are likely to be referred to a pulmonologist — a doctor with training and skills in diagnosing and treating lung disorders.
Pulmonary fibrosis is a serious and complex disease. Take a friend or family member with you to your appointment. That person can take notes, provide emotional support, and help remember information you may miss.
What to Prepare
A list of your symptoms and how long you have had them
Key medical information, including recent hospital stays
Key personal information, such as work that increases your risk or recent travel
All medicines, vitamins, herbs, and supplements, with doses
The questions you want to ask
Questions to Ask
What is likely causing my symptoms?
What tests do I need?
What treatments are recommended?
How will this condition affect my other health problems?
What should I do or not do?
The Doctor May Also Ask You
When did your symptoms start?
Are you receiving treatment for other conditions?
What medicines and supplements have you taken in the past five years?
Do you smoke, and how much?
What type of work have you ever done, even for a few months?
Do any family members have long-term lung disease?
Have you ever had chemotherapy or radiation?
Conclusion: What to Do Next
Pulmonary fibrosis is a serious, progressive lung disease — but it is not untreatable. While the scarring cannot be reversed, treatment can slow the disease, ease breathlessness, and protect your quality of life for as long as possible.
If you have ongoing shortness of breath, a dry cough, or extreme tiredness — especially if you are a middle-aged or older adult with a smoking history, high-risk work exposure, or a family history of lung disease — do not wait it out. Act on these next steps:
Book an appointment with a pulmonologist and describe your symptoms precisely, including how long they have lasted and how fast they are changing.
Write down your exposure history — every job you have held (even briefly), chemical or dust exposures, past radiation or chemotherapy, and your full medicine and supplement list.
Stop smoking now and ask your healthcare team about quitting support; this is the single most important self-care step.
Know the emergency signs: if your breathing suddenly worsens over days or weeks, seek urgent medical care.
Frequently Asked Questions
What is pulmonary fibrosis?
Pulmonary fibrosis is a lung disease in which lung tissue becomes damaged and scarred. The thickened, stiff tissue makes it harder for the lungs to work properly, so less oxygen passes into the bloodstream. It worsens over time, and when no cause can be found it is called idiopathic pulmonary fibrosis.
What are the first signs of pulmonary fibrosis?
The first signs are usually shortness of breath and a dry cough. Extreme tiredness, unintended weight loss, aching muscles and joints, and widening and rounding of the fingertips (clubbing) can also occur. Symptoms progress at very different rates from person to person.
Is pulmonary fibrosis curable?
No. The lung damage cannot be repaired, and no current treatment has proved effective at stopping the disease from worsening. However, medicines such as pirfenidone and nintedanib may slow the progression, and oxygen therapy, pulmonary rehabilitation, and lung transplantation can ease symptoms and improve quality of life.
What causes idiopathic pulmonary fibrosis?
By definition, the cause of idiopathic pulmonary fibrosis is never found. Known risk factors such as smoking or air pollution could be related, and many people with IPF also have GERD (stomach acid reflux), which may be a risk factor or speed the worsening — but more studies are needed.
How long can you live with pulmonary fibrosis?
Progression varies greatly. Some people become very ill quickly with severe disease, while others have moderate symptoms that worsen slowly over months or years. Your healthcare team uses imaging and lung function tests to track your condition and treatment response over time.
When should I go to the emergency room for pulmonary fibrosis?
If your shortness of breath suddenly gets worse over a few days or weeks, this may be an acute exacerbation, which can be life-threatening. Contact your healthcare team right away, and seek emergency care for rapidly worsening breathing, very low oxygen levels, or inability to breathe.
External References
Pulmonary fibrosis — Symptoms & causes — Symptoms, causes, risk factors, and complications (reviewed February 15, 2024)
Pulmonary fibrosis — Diagnosis & treatment — Diagnosis, tests, treatment options, lifestyle, and coping (reviewed February 15, 2024)
References
Health information, not medical advice. This article is for general education and is not a substitute for professional diagnosis or treatment. Always consult a qualified healthcare provider about your own health, and seek emergency care for urgent symptoms.
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