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.

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 are used to treat people with 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?
Pulmonary Medicine | Intensive Care Medicine
Pulmonary Medicine | Intensive Care Medicine

Glens Falls Hospital Inc

100 Park St, 
Glens Falls, NY 
 16.9 mi

Amit Chopra is a Pulmonary Medicine specialist and an Intensive Care Medicine provider in Glens Falls, New York. Dr. Chopra and is rated as an Advanced provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. His top areas of expertise are Lung Metastases, Pneumonia, Lung Cancer, Gastrostomy, and Endoscopy.

Pulmonary Medicine | Intensive Care Medicine
Pulmonary Medicine | Intensive Care Medicine

St. Peter's Health Partners Medical Associates, P.C.

2 New Hampshire Ave, 
Troy, NY 
 24.4 mi

Julio Miranda is a Pulmonary Medicine specialist and an Intensive Care Medicine provider in Troy, New York. Dr. Miranda and is rated as an Experienced provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. His top areas of expertise are Lung Metastases, Chronic Obstructive Pulmonary Disease (COPD), Asthma, and Asthma in Children.

 
 
 
 
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Pulmonary Medicine | Hospital Medicine | Intensive Care Medicine
Pulmonary Medicine | Hospital Medicine | Intensive Care Medicine

Saratoga Hospital

19 W Ave, 
Saratoga Springs, NY 
 1.0 mi

Hung Nguyen is a Pulmonary Medicine specialist and a Hospital Medicine provider in Saratoga Springs, New York. Dr. Nguyen and is rated as an Experienced provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. His top areas of expertise are Excessive Daytime Sleepiness, Obstructive Sleep Apnea, Lung Metastases, Chronic Obstructive Pulmonary Disease (COPD), and Endoscopy.

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/patients-caregivers/medical-and-support-resources/find-a-support-group
  • American Lung Association -- www.lung.org/better-breathers
What is the outlook (prognosis) for Idiopathic Pulmonary Fibrosis?

IPF may improve or stay stable for a long time 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?

Contact 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)
  • Need 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 Phase 2, Multi-Center, Randomized, Double-Blind, Controlled Trial Evaluating the Safety and Efficacy of ENV-101 in Patients With Lung Fibrosis (WHISTLE-PF Trial)

Summary: The goal of this clinical trial is to evaluate the impact that ENV-101 has on lung function and key measures of fibrosis in adult patients with idiopathic pulmonary fibrosis (IPF). Another goal of this study is to better understand the safety and tolerability of ENV-101 in this patient population.

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An Open-label Extension Trial of the Long-term Safety and Efficacy of BI 1015550 Taken Orally in Patients With Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF) (FIBRONEER™-ON)

Summary: This study is open to people with idiopathic pulmonary fibrosis (IPF) or progressive pulmonary fibrosis (PPF). They can only take part if they have completed treatment in a previous study with a medicine called BI 1015550 (study 1305-0014 or 1305-0023). The goal of this study is to find out how well people with pulmonary fibrosis tolerate longterm treatment with BI 1015550. The study also tests wh...

What are the Latest Advances for Idiopathic Pulmonary Fibrosis?
Progressive pulmonary fibrosis (PPF).
The efficacy and safety of pirfenidone in the treatment of HPS-related pulmonary fibrosis and Idiopathic pulmonary fibrosis: a systematic review and meta-analysis.
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Impact of reduction in antifibrotic treatment on mortality in idiopathic pulmonary fibrosis.
Who are the sources who wrote this article ?

Published Date: May 03, 2024
Published By: Allen J. Blaivas, DO, Division of Pulmonary, Critical Care, and Sleep Medicine, VA New Jersey Health Care System, Clinical Assistant Professor, Rutgers New Jersey Medical School, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, 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/idiopathic-pulmonary-fibrosis. Updated June 26, 2023. Accessed May 14, 2024.

Raghu G, Martinez FJ. Interstitial lung disease. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 80.

Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47. PMID: 35486072 pubmed.ncbi.nlm.nih.gov/35486072/.

Ryu JH, Selman M, Lee JS, Colby TV, King TE. Idiopathic pulmonary fibrosis. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 89.

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.