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Pulmonary fibrosis symptoms: What are the early signs of pulmonary fibrosis?

Medically reviewed by Karen Berger, Pharm.D.
Licensed Pharmacist
Updated Oct. 27, 2023  •  Published Sep. 11, 2023
Fact Checked
Pulmonary fibrosis symptoms: What are the early signs of pulmonary fibrosis?

Overview: What does pulmonary fibrosis feel like?

Pulmonary fibrosis is a lifelong medical condition that affects the lungs. It progressively damages and scars the air sacs (alveoli) and connective tissue in the lung, making breathing hard as the scarring progresses and the lung’s tissues grow thicker. People find it hard to draw in a deep breath. To compensate for it, people start breathing fast, like gasping. Early on, they develop a chronic but unproductive cough. As lung function declines, the body doesn’t get enough oxygen, making people tired and fatigued. At first, physical exertion easily wears them out. Eventually, even simple tasks become quickly tiring. Although pulmonary fibrosis worsens over time, some people will experience flare-ups in which the symptoms get very bad quickly. These exacerbations may require hospitalization.

RELATED: What causes shortness of breath? Related conditions and treatments

Key takeaways:

  • Pulmonary fibrosis is an uncommon health condition that can affect anyone regardless of age, sex, race, or ethnicity. But, it’s more common in men and individuals over 50 years old

  • Early signs of pulmonary fibrosis include shortness of breath and persistent dry cough.

  • Serious symptoms of pulmonary fibrosis, such as a rapid worsening of breathing difficulties, may require immediate medical attention.

  • Pulmonary fibrosis is caused by chronic exposure to pollutants or toxic chemicals, radiation therapy to the chest, medication side effects, and certain autoimmune diseases such as rheumatoid arthritis, sarcoidosis, and scleroderma. However, many cases will have no identifiable cause. You may be at higher risk for developing pulmonary fibrosis symptoms if you smoke (or used to smoke), are male, older than 50, or have been regularly exposed to toxic chemicals or pollutants. A family history of pulmonary fibrosis is another significant risk factor.

  • Pulmonary fibrosis requires a medical diagnosis.

  • Pulmonary fibrosis generally requires treatment. Pulmonary fibrosis symptoms temporarily improve with supportive treatment. Drugs are used to slow the progress of the disease.

  • Treatment of pulmonary fibrosis may include supportive care, oxygen therapy, lung rehabilitation therapy, antifibrotic drugs, mechanical ventilation, treatment of complications, and lung transplantation. 

  • Pulmonary fibrosis could result in complications like coronary artery disease, blood clots, lung infections, pneumonia, lung cancer, pulmonary hypertension (high blood pressure in the lungs), GERD, hiatal hernia, obstructive sleep apnea, and respiratory failure.

  • Use coupons for pulmonary fibrosis treatments like Esbriet (pirfenidone) and OFEV (nintedanib) to save up to 80%.

What are the early signs of pulmonary fibrosis?

The early signs of pulmonary fibrosis are difficulty breathing and a persistent dry cough.

Other pulmonary fibrosis symptoms

Pulmonary fibrosis is a progressive condition in which lung tissue becomes damaged and scarred, compromising the ability to breathe and absorb enough oxygen to support the body’s functions. As the disease progresses, symptoms will worsen. The most common symptoms of pulmonary fibrosis are:

  • Difficulty breathing

  • Shortness of breath when exerting oneself or at rest

  • Persistent dry cough

  • Fatigue or tiredness

  • Muscle pain

  • Joint pain

As the disease worsens, other symptoms related to low oxygen levels may appear, such as:

  • Unexplained weight loss

  • Clubbing of the fingers and toes

  • Skin turning blue, gray, or white (cyanosis) from lack of oxygen

Pulmonary fibrosis vs. cystic fibrosis symptoms

Cystic fibrosis is very different from pulmonary fibrosis. Cystic fibrosis is a congenital (inherited) disorder that reduces the water content in body secretions, causing thick mucus to build up. While pulmonary fibrosis mostly affects the lungs, cystic fibrosis affects various organ systems in the body. The two conditions have lung scarring in common, but the process is different. Pulmonary fibrosis scarring is due to inflammation, but people with cystic fibrosis are vulnerable to repeated lung infections due to thick, sticky mucus stuck in their lungs. Over time, the damage and scarring from all these repeated infections accumulate, steadily reducing lung function. Lung disease is the usual cause of death in most individuals with cystic fibrosis. However, people with cystic fibrosis live for an average of 66 years, while about 81% of people with pulmonary fibrosis die within five years of being diagnosed.  

Pulmonary fibrosis Cystic fibrosis
Shared symptoms
  • Lung scarring and associated symptoms
Unique symptoms
  • Most common in older adults
  • Begins at birth
  • Pancreas problems, including pancreatitis, diabetes, and nutritional deficiencies
  • Liver disorders, including fatty liver and cirrhosis
  • Bone problems, including bone loss and osteoporosis
  • Nasal problems such as polyps or swelling due to thick mucus
  • Digestive system problems
  • Infertility

RELATED: Cystic fibrosis treatments and medications

Types of pulmonary fibrosis: How can I tell which one I have? 

Healthcare providers distinguish between interstitial pulmonary fibrosis and idiopathic pulmonary fibrosis. The main difference is what causes the scarring process. Interstitial lung disease has identifiable causes, such as toxins, pollutants, dust, asbestos, infections, radiation treatment, or medication side effects. If there is no known cause, healthcare providers call the disease idiopathic.

When to see a doctor for pulmonary fibrosis symptoms

See a doctor for breathing difficulties or a dry, hacking cough. Pulmonary fibrosis is often misdiagnosed, and many people don’t seek medical help quickly enough. That’s unfortunate because treatment can slow down the progress of the disease, especially in the earliest stages of the disease.

Diagnosis is difficult. Pulmonary fibrosis has many of the same symptoms as other lung diseases and is easily misdiagnosed. People will initially bring their concerns to a family physician. Still, a team of doctors will make the final diagnosis, including a lung specialist (pulmonologist), imaging specialist (radiologist), and pathologist. The process has several steps:

  • A medical history will focus on symptoms and identify causes, such as smoking.

  • Vital signs will help determine oxygen levels in the blood.

  • In a physical exam, the clinician will listen closely to the lungs. Many unusual sounds, such as crackles, may indicate pulmonary fibrosis or other conditions.

  • Blood tests do not help diagnose pulmonary fibrosis but will be used initially to rule out other disorders.

  • Pulmonary function tests will determine your breathing abilities: how much air you can breathe in and how much air can be held in the lungs.

  • A chest X-ray or CT scan will help identify the extent of lung scarring.

  • A bronchoscopy will allow the clinician to view the lungs to directly assess the extent of scarring.

  • A sample of lung tissue may be surgically removed from the body and evaluated by a pathologist. 

Complications of pulmonary fibrosis

Pulmonary fibrosis is associated with a number of comorbidities (having one or more conditions at the same time) and complications, including:

How to treat pulmonary fibrosis symptoms

The treatment of pulmonary fibrosis may involve:

  • At-home supportive treatment options such as supplemental oxygen, breathing exercises, activity, rest, and use of over-the-counter medications to treat symptoms and minor complications such as acid reflux

  • Pulmonary rehabilitation is a set of exercises and breathing techniques overseen by a physical therapist or respiratory therapist to help increase breathing capacity

  • Antifibrotic drugs (pirfenidone or nintedanib) slow the progress of pulmonary fibrosis by blocking the body’s ability to form scar tissue 

  • Treatment of complications such as pulmonary hypertension

  • Lung transplant

Living with pulmonary fibrosis

There is no cure for pulmonary fibrosis. Because it interferes with breathing, it will change the activities people can engage in. Still, it’s possible to manage the condition. The American Lung Association has the following tips for living with pulmonary fibrosis: 

  • Keep all medical appointments

  • Join a pulmonary rehabilitation program

  • Practice breathing exercises as directed

  • Don’t smoke

  • Avoid air pollution or particulates 

  • Use an indoor air purifier

  • Be active every day (check with your healthcare provider about what activities are safe and appropriate)

  • Don’t hesitate to use supplemental oxygen when feeling breathless

  • Eat a healthy diet

  • Reduce stress

  • Join a support group

Most importantly, see a doctor when breathing first becomes a problem

Pulmonary fibrosis is a serious and lifelong illness, but it can be slowed with new types of drugs. These drugs can slow the progression of the disease by 50%. However, too many people go undiagnosed for too long. The Idiopathic Pulmonary Fibrosis Foundation estimates that over half of the people with pulmonary fibrosis do not get diagnosed in the early, milder stages of the illness when treatment can significantly impact life expectancy and quality of life. One-third of those with moderate pulmonary fibrosis go undiagnosed. Almost 1 in 5 people who die of pulmonary fibrosis never get diagnosed. Unfortunately, the early signs of pulmonary fibrosis are subtle and easy to mistake for other problems. A good rule of thumb is this: If you have any persistent symptoms of breathing difficulties or a cough that doesn’t go away after several weeks, talk to a doctor.

FAQs about pulmonary fibrosis symptoms

What does early pulmonary fibrosis feel like?

The earliest signs of pulmonary fibrosis are breathlessness after exertion and a persistent, unproductive cough. 

What are the signs that pulmonary fibrosis is getting worse?

As pulmonary fibrosis progresses, breathing will become more difficult. Because of declining lung function, people will feel more worn out. They may start noticing symptoms of poor oxygenation, particularly clammy or pale skin.

What are the most common late-stage symptoms of pulmonary fibrosis?

As pulmonary fibrosis progresses, symptoms such as breathing difficulties and fatigue worsen. Common late-stage symptoms include clubbing of the fingers and toes and a squeaking sound when drawing in a breath. In the last stages, people will be fatigued. They may sleep much of the time. They’ll also be less interested in daily activities, eating, and drinking.

What’s next? Additional resources for people with pulmonary fibrosis symptoms

Test and diagnostics

Treatments

Scientific studies and clinical trials

Medically reviewed by Karen Berger, Pharm.D.
Licensed Pharmacist

After receiving her doctorate from the University of Pittsburgh School of Pharmacy, Karen Berger, Pharm.D., has worked in both chain and independent community pharmacies. She currently works at an independent pharmacy in New Jersey. Dr. Berger enjoys helping patients understand medical conditions and medications—both in person as a pharmacist, and online as a medical writer and reviewer.

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