Learn About Idiopathic Pulmonary Fibrosis

What is the definition of Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause.

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What are the alternative names for Idiopathic Pulmonary Fibrosis?

Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP

What are the causes of Idiopathic Pulmonary Fibrosis?

Health care providers do not know what causes IPF or why some people develop it. Idiopathic means the cause is not known. The condition may be due to the lungs responding to an unknown substance or injury. Genes may play a role in developing IPF. The disease occurs most often in people between 60 and 70 years old. IPF is more common in men than women.

What are the symptoms of Idiopathic Pulmonary Fibrosis?

When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In most people, IPF gets worse quickly over months or a few years. In others, IPF worsens over a much longer time.

Symptoms may include any of the following:

  • Chest pain (sometimes)
  • Cough (usually dry)
  • Not able to be as active as before
  • Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
  • Feeling faint
  • Gradual weight loss
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What are the current treatments for Idiopathic Pulmonary Fibrosis?

There is no known cure for IPF.

Treatment is aimed at relieving symptoms and slowing disease progression:

  • Pirfenidone (Esbriet) and nintedanib (Ofev) are two medicines that treat IPF. They may help slow lung damage.
  • People with low blood oxygen levels will need oxygen support at home.
  • Lung rehabilitation will not cure the disease, but it can help people exercise with less difficulty breathing.

Making home and lifestyle changes can help manage breathing symptoms. If you or any family members smoke, now is the time to stop.

A lung transplant may be considered for some people with advanced IPF.

Who are the top Idiopathic Pulmonary Fibrosis Local Doctors?
Elite
Highly rated in
9
conditions
Pulmonary Medicine
Intensive Care Medicine

UVA Health System

Pulmonary Clinic

1221 Lee St 
Charlottesville, VA 22903

Imre Noth is a Pulmonary Medicine specialist and an Intensive Care Medicine doctor in Charlottesville, Virginia. Dr. Noth has been practicing medicine for over 30 years and is rated as an Elite doctor by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 9 other conditions, according to our data. His top areas of expertise are Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, Acute Interstitial Pneumonia, and Interstitial Lung Disease. He is licensed to treat patients in Virginia. Dr. Noth is currently accepting new patients.

Elite
Highly rated in
11
conditions
Pulmonary Medicine
Intensive Care Medicine

University of California San Francisco Health System

ILD Program

400 Parnassus Ave 
San Francisco, CA 94143

Paul Wolters is a Pulmonary Medicine specialist and an Intensive Care Medicine doctor in San Francisco, California. Dr. Wolters has been practicing medicine for over 30 years and is rated as an Elite doctor by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 11 other conditions, according to our data. His top areas of expertise are Idiopathic Pulmonary Fibrosis, Acute Interstitial Pneumonia, Pulmonary Fibrosis, and Interstitial Lung Disease. He is licensed to treat patients in California. Dr. Wolters is currently accepting new patients.

 
 
 
 
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Elite
Highly rated in
9
conditions
Pulmonary Medicine
Intensive Care Medicine

Pulmonary Practice At Parnassus

400 Parnassus Ave 
San Francisco, CA 94143

Harold Collard is a Pulmonary Medicine specialist and an Intensive Care Medicine doctor in San Francisco, California. Dr. Collard has been practicing medicine for over 25 years and is rated as an Elite doctor by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 9 other conditions, according to our data. His top areas of expertise are Hypersensitivity Pneumonitis, Acute Interstitial Pneumonia, Interstitial Lung Disease, and Pulmonary Fibrosis. He is licensed to treat patients in California.

What are the support groups for Idiopathic Pulmonary Fibrosis?

You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.

More information and support for people with IPF and their families can be found at:

  • Pulmonary Fibrosis Foundation -- www.pulmonaryfibrosis.org/life-with-pf/support-groups
  • American Lung Association -- www.lung.org/support-and-community/
What is the outlook (prognosis) for Idiopathic Pulmonary Fibrosis?

IPF may improve or stay stable for a longtime with or without treatment. Most people get worse, even with treatment.

When breathing symptoms become more severe, you and your provider should discuss treatments that prolong life, such as lung transplantation. Also discuss advance care planning.

What are the possible complications of Idiopathic Pulmonary Fibrosis?

Complications of IPF may include:

  • Abnormally high levels of red blood cells due to low blood oxygen levels
  • Collapsed lung
  • High blood pressure in the arteries of the lungs
  • Respiratory failure
  • Cor pulmonale (right-sided heart failure)
  • Death
When should I contact a medical professional for Idiopathic Pulmonary Fibrosis?

Call your provider right away if you have any of the following:

  • Breathing that is harder, faster, or shallower (you are unable to take a deep breath)
  • To lean forward when sitting to breathe comfortably
  • Frequent headaches
  • Sleepiness or confusion
  • Fever
  • Dark mucus when you cough
  • Blue fingertips or skin around your fingernails
Spirometry
Clubbing
Respiratory system
What are the latest Idiopathic Pulmonary Fibrosis Clinical Trials?
A Randomized, Double-Blind, Placebo-Controlled Phase II Clinical Trial of GKT137831 in Patients With Idiopathic Pulmonary Fibrosis
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Use of the Src Family Kinase Inhibitor Saracatinib in the Treatment of Idiopathic Pulmonary Fibrosis
What are the Latest Advances for Idiopathic Pulmonary Fibrosis?
Antacid Medication and Antireflux Surgery in Patients with Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
Evaluation for clinical benefit of metformin in patients with idiopathic pulmonary fibrosis and type 2 diabetes mellitus: a national claims-based cohort analysis.
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Safety and tolerability of nintedanib in patients with progressive fibrosing interstitial lung diseases: data from the randomized controlled INBUILD trial.
Who are the sources who wrote this article ?

Published Date : January 01, 2020
Published By : Denis Hadjiliadis, MD, MHS, Paul F. Harron, Jr. Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

National Heart, Lung, and Blood Institute website. Idiopathic pulmonary fibrosis. www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis. Accessed January 13, 2020.

Raghu G, Martinez FJ. Interstitial lung disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 86.

Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3-e19. PMID: 26177183 pubmed.ncbi.nlm.nih.gov/26177183/.

Ryu JH, Selman M, Colby TV, King TE. Idiopathic interstitial pneumonias. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 63.

Silhan LL, Danoff SK. Nonpharmacologic therapy for idiopathic pulmonary fibrosis. In: Collard HR, Richeldi L, eds. Interstitial Lung Disease. Philadelphia, PA: Elsevier; 2018:chap 5.