Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that worsens over time and has no known cure. It causes scarring in the lungs, which thickens the lung tissue and makes it harder for oxygen to pass into the bloodstream. This damage is permanent, making it increasingly difficult to breathe. While some medications may help slow the progression of the disease, they cannot reverse the damage that has already occurred. In certain cases, doctors may recommend a lung transplant to improve a person’s chances of survival.
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What Is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis (IPF) is a disease marked by progressive scarring of the lungs. The disease is called idiopathic because its cause is unknown. IPF is the most common type of pulmonary fibrosis.
Fibrosis, or scar tissue, stiffens the tissue lining the lungs and impairs their ability to transport oxygen. This stiffening gradually interferes with a person’s ability to breathe.
IPF belongs to a family of more than 200 related diseases called interstitial lung diseases (ILDs), which often have similar characteristics and can result in lung scarring. Lung damage from IPF is irreversible and progressive, meaning it can’t be undone, and it gets worse over time.
How common is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis (IPF) is the most common type of pulmonary fibrosis.
Between 2017 and 2022, the incidence rate of IPF cases (number of new cases) ranged from 13 to 26 per 100,000 people, depending on the definition of IPF. The total number of people living with IPF ranged from 44 to 89 per 100,000. New cases stayed about the same each year, but the total number of people with IPF grew steadily, from 37.5 per 100,000 in 2017 to 96.2 per 100,000 in 2022.
What causes idiopathic pulmonary fibrosis?
IPF belongs to a group of conditions known as interstitial lung diseases (ILDs). It develops when lung tissue becomes abnormally thick and rigid, eventually leading to scarring deep within the lungs. This fibrosis (scarring) appears to stem from a repeated pattern of injury and repair, where the normal healing response becomes disrupted. Instead of restoring healthy tissue, the lungs begin to lay down scar tissue. The underlying trigger for this faulty repair process remains unclear.
Idiopathic pulmonary fibrosis risk factors
- Age — IPF is rare before age 50: Fewer than 10% of patients are under 65. About 60% are age 75 or older.
- Environmental exposures — Possible links to air pollution, viral infections, workplace exposures (e.g., dust, chemicals).
- Family history of pulmonary fibrosis — Having relatives with IPF raises your risk.
- Genetic factors — Certain genes are linked to increased risk, including: MUC5B, TERT, TERC, DKC1, RTEL1, AKAP13, DSP, FAM13A, DPP9, and TOLLIP.
- Race — Rates are higher among white and Hispanic groups than Black and Asian populations.
- Smoking history — Current and former smokers have a higher chance of developing IPF than those who never smoked.
Most people with IPF have other serious chronic health issues, including gastroesophageal reflux disease (GERD) and sleep apnea. Only 3% of the general population of people without IPF have the same level of health problems as those with IPF.
Complications of idiopathic pulmonary fibrosis
Complications of untreated IPF may include:
- Depression and anxiety.
- Lung cancer.
- Pulmonary hypertension.
- Respiratory failure.
- Sleep problems or disorders.
Ultimately, treated or untreated, IPF is eventually fatal.
How can I prevent idiopathic pulmonary fibrosis?
Currently, there’s no way to predict who might develop IPF in advance. But if you have factors that raise your risk — such as if someone in your family has had it – your doctor might suggest doing a lung function test or a high-resolution chest CT scan to check for any scarring in your lungs.
What Are the Signs and Symptoms of Idiopathic Pulmonary Fibrosis?
IPF, as with other interstitial lung diseases, affects each person differently and at different rates.
Typical early symptoms of IPF include:
- Dry cough.
- Shortness of breath during periods of activity.
Other possible symptoms include:
- Fatigue.
- Unexplained weight loss.
Later-stage symptoms include:
- Enlargement or clubbing of the fingertips or toes may develop. Clubbing is a widening and rounding of the tips of the fingers or toes.
- As the disease progresses, shortness of breath may occur at rest, making it hard to do even normal activities — such as walking, talking on the phone, and eating.
When should I see a doctor about my idiopathic pulmonary fibrosis symptoms?
If you experience these symptoms, talk to your health care provider. They may order tests to help rule out other types of idiopathic pulmonary fibrosis.
- Shortness of breath that gets worse over time
- Dry cough that doesn’t improve.
- Muscle and joint aches.
- Feeling tired or weak.
- Losing weight slowly without trying.
How Do You Diagnose Idiopathic Pulmonary Fibrosis?
Diagnosing interstitial lung diseases (ILDs) can be challenging, as it often requires doctors to piece together a wide range of clinical data. In some cases, the underlying cause is apparent, making the diagnosis more straightforward. But, for others, the origin of their lung issues remains unclear, and their symptoms don’t align neatly with any particular ILD subtype.
IPF is the most recognized form of ILD without a known cause, but it’s far from the only one — many ILDs are labeled idiopathic because their root causes are still unknown to medical science.
Medical history and physical exam
Your doctor will start by reviewing your symptoms, family and medical history, and risk factors, and then conduct a physical examination.
Your doctor may look for signs of IPF during a physical exam, such as:
- Blue hands and feet from lack of oxygen in the blood.
- Clubbing (widening at the tips) of the fingers or toes.
- High-pitched, crackly sounds when listening to your lungs.
Your doctor may order a few tests to rule out other possible medical causes of your lung issues. Idiopathic means that a cause for your disease cannot be determined at the time of diagnosis.
Tests to diagnose idiopathic pulmonary fibrosis
To help confirm a diagnosis of IPF, UPMC specialists conduct several tests, including:
- Breathing tests — To show how the lungs are working.
- High-resolution CT scans of the chest — To provide a more detailed picture of the lungs.
- Oxygen saturation tests — To check oxygen levels at rest, with exertion, and during sleep.
Additional procedures may include:
- Echocardiogram — To assess any heart involvement. As a result of low oxygen levels, the heart may be stressed and cause pulmonary hypertension (high blood pressure in the lungs).
- Lab tests, including rheumatologic studies — To exclude other causes of lung scarring.
- Lung biopsy or video-assisted thoracic (VAT) surgery — To find usual interstitial pneumonia, the histological (what the tissue looks like under a microscope) diagnosis for IPF.
Idiopathic pulmonary fibrosis prognosis
The prognosis for IPF is generally poor. More than half of people with IPF were initially misdiagnosed. After diagnosis, 50% of people with IPF will die within two years, and 80% will die within five years.
How Do You Treat Idiopathic Pulmonary Fibrosis?
At UPMC, a multidisciplinary team of experts offers people with IPF the full capabilities of UPMC. Patients are invited for an evaluation and ongoing management of their diseases in conjunction with their local doctors. Researchers and clinicians collaborate to translate basic research on ILDs into new treatments that may save lives.
Nonsurgical treatments for IPF
Currently, there are no approved drug therapies for IPF.
However, people with IPF may benefit from:
- Going to pulmonary rehabilitation.
- Joining an IPF support group.
- Monitoring for acute flare-ups.
- Participating in clinical research.
- Using oxygen therapy.
People with IPF may also receive referral to UPMC for lung transplant services, if necessary.
Surgical treatments for IPF
Lung transplantation is the only effective treatment for IPF and is considered an option in the management of IPF for most patients.
UPMC's lung transplantation services are nationally renowned. Our experts have performed more than 1,400 lung transplants since the program’s inception.
Why Choose UPMC for Idiopathic Pulmonary Fibrosis Care?
At UPMC, the multidisciplinary idiopathic pulmonary fibrosis treatment team includes:
- Pulmonary physicians — Experts in the management of IPF
- Cardiologists — Evaluate the involvement of the heart and pulmonary vessels
- Rheumatologists — Assess the immune system
- Pathologists — Evaluate the biopsies
- Chest radiology experts — Evaluate imaging results
- Rehabilitation and quality-of-life experts — Optimize supportive therapy and assess the psychological impact of IPF
- Transplant surgeons – Perform lung transplants for people with IPF.
- Leaders in clinical research — who provide access to novel IPF therapies
The team will re-evaluate the diagnosis and assess treatment strategies once the diagnosis is confirmed.
