Pulmonary Fibrosis: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Pulmonary Fibrosis?
A pulmonologist, which is a doctor who treats lung diseases, is the type of doctor who usually diagnoses and treats pulmonary fibrosis. Some people may also discuss a lung transplant with their provider. 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 pulmonary fibrosis?
Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This tissue gets thick and stiff. That makes it hard for you to catch your breath, and your blood may not get enough oxygen.
In most cases, the cause cannot be found. This is called idiopathic pulmonary fibrosis, or IPF.
Symptoms
Symptoms of pulmonary fibrosis include:
- Shortness of breath.
- A dry, hacking cough that does not get better.
- Fatigue, which means feeling very tired.
- Weight loss for no known reason.
- Aching muscles and joints.
- Clubbing, which is the widening and rounding of the tips of the fingers or toes.
In IPF, shortness of breath during activity lasts for months or years. Over time, it may also occur when you are at rest. With advanced disease, the skin around the mouth or fingernails can turn bluish because of low oxygen.
In most people with IPF, the disease gets worse quickly over months or a few years. In others, it gets worse over a much longer time.
Causes and who gets it
Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs.
For IPF, health care providers do not know what causes it or why some people develop it. Genes may play a role. IPF occurs most often in people between 60 and 70 years old. It is more common in men than in women.
How doctors diagnose it
Your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. A biopsy means that a tissue sample is taken from the lung so it can be examined.
For IPF, your provider will ask whether you have been exposed to asbestos or other toxins and whether you have been a smoker. The exam may find abnormal breath sounds called crackles, which sound like Velcro being pulled apart.
A high resolution chest CT scan takes pictures of the inside of your lungs and looks for scarring or inflammation. For IPF, doctors look for lungs that look like a honeycomb. Lung function tests show how well you breathe and how much oxygen is in your blood. Doctors also rule out other conditions that may be causing your symptoms. For IPF, tests for autoimmune diseases such as rheumatoid arthritis, lupus, or scleroderma may also be done.
Treatment
There is no cure. Treatments can help with symptoms and improve your quality of life. They include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant.
For IPF, pirfenidone, nintedanib, and nerandomilast are medicines used for treatment. They help slow lung damage but do not reverse damage that has already occurred. People with low blood oxygen levels benefit from supplemental oxygen. Lung rehabilitation will not cure the disease, but it can help people exercise with less difficulty breathing.
A lung transplant is an option for some people with pulmonary fibrosis. If you or any family members smoke, now is the time to stop.
IPF may improve or stay stable for a long time with or without treatment. Most people get worse, even with treatment. For IPF, possible complications include a collapsed lung, high blood pressure in the arteries of the lungs, and respiratory failure.
When to see a doctor
For IPF, the source says to contact your provider right away if you have any of these signs:
- Breathing that is harder, faster, or shallower, so you are unable to take a deep breath.
- The need to lean forward when sitting to breathe comfortably.
- Fever, or dark mucus when you cough.
- Frequent headaches or sleepiness.
Call 911 if you have severe trouble breathing, chest pain or discomfort lasting two minutes or more, fainting, 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. Either kind of doctor can talk through your symptoms, help set up visits with a lung specialist, and keep track of your care over time.
Common questions
Can pulmonary fibrosis be cured?
No, there is no cure for pulmonary fibrosis. Treatments can help with symptoms and improve your quality of life.
What is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis is pulmonary fibrosis with no cause that doctors can find. In most cases of pulmonary fibrosis, the cause cannot be found, and this is called idiopathic pulmonary fibrosis.
Does pulmonary fibrosis cause shortness of breath at rest?
It can, over time. In IPF, shortness of breath during activity lasts for months or years, and over time it may also occur when you are at rest.
Is a lung transplant an option for pulmonary fibrosis?
Yes, a lung transplant is an option for some people with pulmonary fibrosis. When breathing symptoms become more severe, you and your provider should discuss treatments that prolong life, such as lung transplantation.
Sources
- MedlinePlus - Pulmonary Fibrosis
- MedlinePlus - Idiopathic pulmonary fibrosis
- National Heart, Lung, and Blood Institute - Pulmonary Fibrosis Diagnosis
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.
