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 eventually 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), nintedanib (Ofev), and nerandomilast (Jascayd) are medicines that are used to treat people with IPF. They help slow lung damage but do not reverse damage that has already occurred.
- People with low blood oxygen levels benefit from supplemental oxygen.
- 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 is an option for some people with pulmonary fibrosis.
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.
Baylor College Of Medicine - Pulmonary
Ivan Rosas, MD, is dedicated to delivering high-quality, compassionate care to Houston and nearby communities. Ivan Rosas specializes in Pulmonary Disease and works at BCM - Faculty Group Practice. To make an appointment, please call (713) 798-2400. To review all accepted insurance carriers, please visit: https://www.stlukeshealth.org/patients-visitors/patients/billing-insurance/insurances-accepted. Dr. Rosas 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 Pulmonary Fibrosis, Acute Interstitial Pneumonia, Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis, and Lung Transplant. Dr. Rosas is currently accepting new patients.
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/help-support/better-breathers-club
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: This study is a prospective observational study for subjects with idiopathic pulmonary fibrosis (IPF) or non-IPF interstitial lung diseases (ILD). The purpose of this study is to compare whether imaging patterns from high-resolution computed tomography (HRCT) at baseline can predict worsening. Single Time point Prediction (STP) is a score derived from an artificial intelligenc/ machine learning (A...
Summary: This is a Ph 2, randomized, double-blind, placebo-controlled global multicenter study to evaluate the efficacy, safety, tolerability, and pharmacokinetics (PK) of PIPE-791 in subjects with a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) with or without background treatment.
Published Date: May 04, 2026
Published By: Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. 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. What is idiopathic pulmonary fibrosis? www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis. Updated June 26, 2023. Accessed June 10, 2026.
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.