Idiopathic Pulmonary Fibrosis: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis is usually treated by a pulmonologist, a doctor who specializes in lung diseases. Your care may also include lung rehabilitation, a program that helps you exercise with less trouble breathing. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause. The word idiopathic means the cause is not known. IPF is a serious long-term disease that affects the tissue around the air sacs in the lungs. Over time, the scarring can become permanent and make it harder and harder to breathe.
IPF belongs to a group of lung diseases that involve swelling or scarring in the lung. About 100,000 people in the United States have it.
Symptoms
Some people may not have symptoms at first. Symptoms can appear later and get worse as the disease grows.
- Shortness of breath during activity, which may also happen at rest over time.
- A cough that is usually dry.
- Not being able to be as active as before.
- Gradual weight loss.
- Chest pain, sometimes.
- Widened and rounded tips of the fingers and toes, called clubbing.
- Bluish skin around the mouth or fingernails when the disease is advanced, because of low oxygen.
In most people, IPF gets worse over months or a few years. In others, it worsens over a much longer time. Some people have sudden times when symptoms become much more serious, called acute exacerbations.
Causes and who gets it
Health care providers do not know what causes IPF or why some people develop it. Genes may play a role. Your risk is higher if you smoke or have a family history of IPF, and the risk goes up with age. The disease usually affects people between the ages of 50 and 70. IPF is more common in men than women.
Researchers have looked at several things in the environment, including wood or metal dust, viral infections, certain medicines, and cigarette smoking. Some research suggests that acid reflux, also called GERD, may also be a risk factor. In most cases, only one person in a family has IPF.
How doctors diagnose it
Your provider will do a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos or other toxins and if you have been a smoker. The exam may find abnormal breath sounds called crackles, which sound like Velcro being pulled apart. Tests that help diagnose IPF include:
- A chest x-ray.
- A high resolution chest CT scan.
- Pulmonary function tests.
- A 6-minute walk test.
- Measurements of the oxygen level in your blood.
- Tests for autoimmune diseases such as rheumatoid arthritis, lupus, or scleroderma.
- Bronchoscopy, an echocardiogram, or an open lung (surgical) biopsy.
Treatment
There is currently no cure for IPF. However, certain treatments may slow the disease and help your lungs work better. Treatment is aimed at relieving symptoms and slowing disease progression.
- Pirfenidone (Esbriet), nintedanib (Ofev), and nerandomilast (Jascayd) are medicines used to treat people with IPF.
- These medicines help slow lung damage but do not reverse damage that has already occurred.
- People with low blood oxygen levels benefit from extra oxygen.
- Lung rehabilitation will not cure the disease, but it can help people exercise with less difficulty breathing.
- If you or any family members smoke, now is the time to stop.
- A lung transplant is an option for some people.
IPF may improve or stay stable for a long time with or without treatment. Most people get worse, even with treatment.
When to see a doctor
Contact your provider right away if you have frequent headaches, fever, or dark mucus when you cough.
Call 911 or go to the emergency room if you have severe trouble breathing, such as breathing that is harder or faster so that you cannot take a deep breath, or a need to lean forward to breathe. Also call 911 for chest pain that lasts two minutes or more, fainting, sleepiness or confusion, or blue fingertips or blue skin around your fingernails.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. When breathing symptoms become more severe, you and your doctor should discuss treatments that prolong life, such as lung transplantation.
Common questions
Is there a cure for idiopathic pulmonary fibrosis?
No, there is currently no cure for IPF. Some treatments may slow the disease and help your lungs work better.
What does idiopathic mean?
Idiopathic means the cause is not known. Health care providers do not know what causes IPF or why some people develop it.
Who gets idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis usually affects people between the ages of 50 and 70. It is more common in men than women. Smoking and a family history of IPF also raise the risk.
Does idiopathic pulmonary fibrosis always get worse quickly?
No, the way IPF progresses is different from person to person. In some people, the disease stays the same for years. In other people, the condition quickly gets worse.
Can idiopathic pulmonary fibrosis cause other health problems?
Yes, IPF can lead to other problems, including pulmonary hypertension and respiratory failure. Pulmonary hypertension means high blood pressure in the arteries of the lungs. Some people also develop lung cancer, blood clots in the lungs, or pneumonia.
Sources
- National Heart, Lung, and Blood Institute - Pulmonary Fibrosis: What Is Idiopathic Pulmonary Fibrosis?
- MedlinePlus - Idiopathic pulmonary fibrosis
- MedlinePlus Genetics - Idiopathic pulmonary fibrosis
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
