Chronic Care

Idiopathic pulmonary fibrosis treatment available today

Idiopathic pulmonary fibrosis, or IPF, is a rare disease that is not curable at this time, however, the progress may be slowed and symptoms reduced with appropriate treatment. Our board-certified primary care online doctors can provide a short-term refill of a medication you are already receiving from your in-person lung specialist so that you do not run out of your medication. This does not replace the necessary in-person care and management with a lung specialist (Pulmonologist).

Written by PlushCare Content Team Last updated: 05/20/2025

Medically reviewed by Jessica Saberman, MD on 04/15/2024

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Learn about idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a lung disease most commonly affecting people:

50 and over
More common in men than women
More common in people who smoke (or used to smoke)
People with a parent or sibling who has IPF

This lung disease makes it difficult for the lungs to properly transport oxygen into the bloodstream.

IPF patients are also at slightly higher risk of lung cancer. There is no cure, nor is the damage done to the lungs irreversible. However, there are forms of IPF treatment available to help ease the symptoms. Most patients with IPF live between 3 to 5 years after diagnosis.

Idiopathic pulmonary fibrosis causes

A build-up of scar tissue around the small breathing sacs in the lungs called alveoli causes idiopathic pulmonary fibrosis (IPF). This scarring makes it difficult for oxygen to pass from the lungs into the bloodstream. The exact cause of this IPF and other forms of interstitial lung disease is unknown, but it is believed to be caused by a combination of genetic and environmental factors. IPF is one form of interstitial lung disease (ILD). Some forms of ILD are more common in people living with Rheumatoid Arthritis or similar autoimmune arthritis conditions.

Symptoms of idiopathic pulmonary fibrosis

The most common symptom of lung fibrosis is shortness of breath. Other symptoms may include:

A persistent cough

Fatigue

Weight loss

Chest pain

Joint pain

Clubbing of the fingers or toes

How to treat idiopathic pulmonary fibrosis

There is no cure for patients with idiopathic pulmonary fibrosis, but there are treatments that can help to improve your symptoms and quality of life. Treatment options include:

Pulmonary rehabilitation: This program of exercise and education can help improve your breathing and overall fitness.
Oxygen therapy: This involves using supplemental oxygen to help you breathe more easily, and is only used for those with hypoxia (abnormally low oxygen levels).
Medications: There are several different medicines that can be used to treat idiopathic pulmonary fibrosis, including anti-inflammatory drugs, immunosuppressants, and antifibrotic drugs. These are almost always managed by lung specialists (Pulmonologists) who are best able to discuss treatment options designed for people living with IPF.
Lung transplants: In some cases, a lung transplant may be a final option. This is a major surgery with a long recovery time, but it can improve your symptoms and quality of life.
Clinical trials: There are often trials for new idiopathic pulmonary fibrosis treatments. Ask your pulmonologist if there are local clinical trials that you may be eligible for.

Idiopathic pulmonary fibrosis medications

There are a variety of medications that physicians can use to treat idiopathic pulmonary fibrosis. These medications can help relieve symptoms and improve the quality of life for IPF patients. Medications used to treat idiopathic pulmonary fibrosis include:

Corticosteroids

These medications can help to reduce inflammation, reduce chest tightness and achy joints, and improve pulmonary function.

Prednisone

Dexamethasone

Anti-fibrotic medicines

Anti-fibrotic medicines that are FDA-approved for the treatment of IPF:

Pirfenidone (Esbriet, Pirfenex, Pirespa): This medication is used to slow the progression of idiopathic pulmonary fibrosis. It is taken orally three times daily.

Nintedanib (Ofev): This medication is used to slow the progression of idiopathic pulmonary fibrosis. It is taken orally twice daily. It should be noted that it has a known bleeding risk and commonly causes severe diarrhea.

Immunosuppressant drugs

These medications can help reduce the immune system's activity and potentially slow the progression of IPF.

Mycophenolate mofetil (CellCept)

Azathioprine (Imuran)

Acid Reducing drugs

Proton pump inhibitors such as omeprazole (Prilosec) or lansoprazole (Prevacid) can help reduce acid reflux from causing damage by dripping into the lungs and are sometimes used to help reduce cough symptoms.

When to see a doctor for idiopathic pulmonary fibrosis

If you are experiencing any symptoms of idiopathic pulmonary fibrosis, it is important to see your doctor. Early diagnosis and treatment are essential for the best possible outcome.

You must see your doctor regularly for checkups if you have been diagnosed with idiopathic pulmonary fibrosis. Your doctor will monitor your symptoms and the progression of the disease. Forced vital capacity (FVC) is a measure to monitor the lung function of patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis treatment FAQs

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease. It is characterized by the buildup of scar tissue in the lungs, making it difficult for oxygen to pass into the bloodstream.

The exact cause of IPF is unknown, but it is believed to be caused by a combination of genetic and environmental factors. The American Lung Association, The American Thoracic Society, and Pulmonary Fibrosis Foundation are excellent sources of information for overall lung health-related quality content. It is also a great source of patient education material.

What are the first signs of IPF?

IPF causes slowly worsening respiratory problems, such as shortness of breath, a dry cough, shallow breathing, gradual weight loss, tiredness, and aching joints. Many of these symptoms can be seen in other conditions; however, the continuous worsening of these despite decongestants and inhaled medications symptoms distinguishes IPF from the common cold, asthma or even COPD/ emphysema.

What is used to treat IPF?

Once scar tissue has formed, it is irreversible. There is no cure for IPF, but treatments can help slow the disease's progress, such as Kinase inhibitors Nintedanib (Ofev) and Pirfenidone (Esbriet, Pirfenex, Pirespa).

These medications aid in slowing the scarring (fibrosis) caused by IPF. They may also assist with preventing an unexpected and striking worsening of symptoms over a few days or weeks, allowing patients treated to live longer. These are the only medications officially approved by the FDA specifically for the treatment of IPF.

How long can you live after being diagnosed with pulmonary fibrosis?

The average survival time following a diagnosis of IPF patients is 3 to 5 years. Keep in mind that this is an average and that some individuals survive much longer than five years with IPF. With early diagnosis and appropriate management, many people live normal lives for years after being diagnosed.

How is idiopathic pulmonary fibrosis diagnosed?

IPF is typically diagnosed through a combination of tests, including a physical examination, a chest X-ray, pulmonary function tests, heart tests such as an echocardiogram, blood work, and a CT scan. A bronchoscopy or a lung biopsy may also be performed to confirm the diagnosis.

What are the stages of idiopathic pulmonary fibrosis?

There are four stages of IPF disease progression, each with its own set of symptoms:

Stage 1: Mild symptoms, such as shortness of breath and a dry cough
Stage 2: Moderate symptoms, such as fatigue, weight loss, and difficulty breathing
Stage 3: Severe symptoms, such as respiratory failure and heart failure
Stage 4: End-stage disease, which is fatal

What are the risk factors for IPF?

IPF is a progressive disease, meaning it gets worse over time. The rate of progression can vary from person to person. IPF can lead to lung failure and death.

The exact cause of IPF is unknown; however, certain risk factors may increase your risk of developing the disease, such as:

Age: IPF is most commonly diagnosed in people between 50 and 70.
Gender: IPF is more common in men than women.
Smoking: Smoking is the most significant risk factor for IPF.
Family history: People with a family history of IPF are more likely to develop the disease.
Environmental factors: Exposure to certain airborne toxins, such as those found in certain occupations (e.g., farming, construction) or hobbies (e.g., woodworking), may increase your risk of IPF.

What are the complications of IPF?

Complications of IPF can include:

Respiratory failure: This is the most common complication of IPF. Respiratory failure occurs when the lungs cannot adequately exchange oxygen and carbon dioxide.
Heart problems: IPF can strain the heart and may cause heart failure.
Kidney problems: IPF can cause kidney failure in some people.
Depression: IPF can cause depression and anxiety.
Infections: Patients with IPF are at an increased risk of developing infections, such as pneumonia.

Step 1

Book an appointment.

Book a same day appointment from anywhere.

Step 2

Talk to your board certified primary care doctor regarding your IPF symptoms and current treatment plan.

Visit with a doctor on your smartphone or computer.

Step 3

Pick up a prescription for IPF treatment, if deemed appropriate by your virtual primary care doctor.

We can send prescriptions to any local pharmacy.

Related conditions to idiopathic pulmonary fibrosis

There are several conditions that are related to idiopathic pulmonary fibrosis. These include:

Bronchiectasis is a condition in which the lungs' airways are damaged and widened. This can lead to difficulty breathing and an increased risk of infection.
Chronic obstructive pulmonary disease (COPD) is a group of conditions that cause airflow obstruction in the lungs. COPD can make breathing difficult and lead to coughing, wheezing, and chest pain.
Pulmonary hypertension is a condition in which the blood pressure in the lungs increases. This can cause shortness of breath, fatigue, and lightheadedness.
Sarcoidosis is a condition in which inflammation occurs in the lungs and other organs. Sarcoidosis can cause shortness of breath, coughing, and fatigue.

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