Idiopathic pulmonary fibrosis


What is Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). IPF is the most common and most lethal type of interstitial lung disease (ILD).

How common is Idiopathic Pulmonary Fibrosis

The estimated prevalence of IPF in the United States varies between 14 and 27.9 cases per 100,000 of the population, while incidence of IPF in the U.S. is estimated at 6.8 to 8.8 cases per 100,000.1 IPF is diagnosed more commonly in men vs. women and most commonly diagnosed in patients age 75 or older. Idiopathic Pulmonary Fibrosis newly occurs in approximately 12 per 100,000 people per year.

Questions regarding your Idiopathic Pulmonary Fibrosis therapy

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  • 1Luba Nalysnyk, Javier Cid-Ruzafa, Philip Rotella, Dirk Esser. European Respiratory Review Dec 2012, 21 (126) 355-361; DOI: 10.1183/09059180.00002512


What are the symptoms of Idiopathic Pulmonary Fibrosis

Symptoms of IPF may include:

  • Exertional dyspnea
  • Labored breathing
  • Dry cough
  • Muscle and joint aches
  • Loss of energy (also called fatigue)
  • Loss of appetite
  • Weight loss
  • Clubbing (rounding of the tips of the fingers or toes)

How is it diagnosed

The diagnosis of idiopathic pulmonary fibrosis can be challenging. Signs and symptoms can be very similar to other interstitial lung disease disorders. Additionally, other disease states can display signs and symptoms similar to IPF, therefore ruling out other disorders is essential before making a diagnosis.

Blood tests

Blood tests can detect oxygen or carbon dioxide level abnormalities that could assist in the diagnosis of IPF.

Imaging tests

A chest radiograph (X-ray) can be used to assist in the diagnosis, although it is not diagnostic on its own. Chest radiographs are low sensitivity and would show lower lobe volume loss in more advanced disease states. A High-resolution computed tomography (HRCT) is the gold standard for IPF diagnosis. It has excellent specificity and can diagnosis IPF through the presence of “honeycombing” patterns in the lungs.

Pulmonary function tests

These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause. Specific pulmonary function tests include Spirometry and diffusion capacity and Oximetry.

Lung tissue analysis

Tissue samples of a patient’s lung can be helpful in determining diagnosis of idiopathic pulmonary fibrosis. Bronchoscopy, bronchoalveolar lavage, and surgical biopsy are particular ways in which tissue samples can be collected.

High resolution computerized tomography

This imaging test is key to, and sometimes the first step in, the diagnosis of idiopathic pulmonary fibrosis. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by IPF. It can show details of the fibrosis, which can be helpful in narrowing down the diagnosis of IPF, which is characterized as a “honeycombing” pattern.


Idiopathic Pulmonary Fibrosis (IPF) Medications

While there is currently no cure for IPF, different treatment options are available to help you manage your disease and feel better day-to-day. Your doctor may prescribe other medication(s) as part of your treatment based on your particular symptoms and level of severity. The following specialty medication is available at Accredo for the treatment of IPF.

Medication Manufacturer
Esbriet® (pirfenidone) Multiple Manufacturers
Ofev® (nintedanib) Boehringer Ingelheim Pharmaceuticals, Inc.

Financing Your Care

Financial assistance coordination may be available to help with your medication costs, including manufacturer and community programs. Accredo representatives are available to help find a program that may work for you.

Community financial resources

Financial Guidance

Life-saving specialty medication can be expensive. Learn how the Accredo teams help individuals find ways to afford the medication they need to survive in this video.

Find Support

There are organizations that support research and advocacy for pulmonary fibrosis. See below for a few of those organizations.

Support organizations

Government organizations

Meet the Team

Accredo’s Idiopathic pulmonary fibrosis care team is dedicated to serving you and we understand the complexity of your condition. Our specialty-trained clinicians are available 24 hours a day, seven days a week, to answer any questions.

Why We Do It

Accredo supports patients with chronic and complex conditions and helps them live their best life. Watch our video to learn why we do what we do for our patients.

Why We Do It video transcript

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