Idiopathic Pulmonary FibrosisSymptoms, Doctors, Treatments, Advances & More
Learn About Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause.
Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
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.
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
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.
Taubman Center
Director of the Michigan Medicine Interstitial Lung Disease program, Vice-Chair of the Medical School Institutional Review Board for Human Subjects Research, Associate Program Director Internal Medicine Residency Program - Research, Co-Director University of Michigan Medical School Scientific Discovery Pathway, Steering committee chair for the pulmonary fibrosis foundations Care Center Network and Patient Registry. Dr. Flaherty is rated as an Elite provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 13 other conditions, according to our data. His clinical expertise encompasses Acute Interstitial Pneumonia, Interstitial Lung Disease, Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, and Lung Transplant. Dr. Flaherty is board certified in Pulmonary Disease.
UPMC Comprehensive Lung Center - Simmons Center
Kevin Gibson, MD, specializes in interstitial lung disease and is board-certified in pulmonary disease and internal medicine by the American Board of Internal Medicine. He practices at UPMC Comprehensive Lung Center - Simmons Center and is affiliated with University of Pittsburgh Physicians, Department of Medicine and UPMC Presbyterian. Dr. Gibson completed his medical degree at University of Medicine and Dentistry of New Jersey, residency at Emory University School of Medicine, and fellowship at the University of Pittsburgh School of Medicine.Dr. Gibson specializes in treating patients with idiopathic pulmonary fibrosis, sarcoidosis, autoimmune lung disease, and occupational lung disease. Dr. Gibson is rated as an Elite provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 11 other conditions, according to our data. His clinical expertise encompasses Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, Acute Interstitial Pneumonia, Interstitial Lung Disease, and Lung Transplant. Dr. Gibson is board certified in American Board Of Internal Medicine and American Board Of Internal Medicine. Dr. Gibson is currently accepting new patients.
Temple University Hospital
Gerard Criner is an Intensive Care Medicine provider practicing medicine in Philadelphia, Pennsylvania. Dr. Criner is rated as an Elite provider by MediFind in the treatment of Idiopathic Pulmonary Fibrosis. He is also highly rated in 33 other conditions, according to our data. His clinical expertise encompasses Chronic Obstructive Pulmonary Disease (COPD), Emphysema, Bronchitis, Lung Transplant, and Embolectomy.
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
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.
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
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
Summary: MKC-NI-002 is a Phase 1b, randomized, double-blind, placebo-controlled study of nintedanib inhalation powder (MNKD-201) in patients with Idiopathic Pulmonary Fibrosis (IPF). The trial consists of Multiple Ascending Doses (MAD) with the primary objective to evaluate safety, tolerability and pharmacokinetics (PK) of MNKD-201 compared to placebo in patients with IPF.
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 nerandomilast or BI 1015550. The goal of this study is to find out how well people with pulmonary fibrosis tolerate long- term treatment with nerandomilast. The study also tests whether ner...
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.
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.