BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuIdiopathic pulmonary fibrosisIdiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIPIdiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause. Causes 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. Symptoms 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) Shortness of breathBreathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough airImageRead Article Now Book Mark Article Feeling faint Gradual weight loss Exams and Tests The provider will do a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos or other toxins and if you have been a smoker.The physical exam may find that you have:Abnormal breath sounds called crackles Bluish skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease) Enlargement and curving of the fingernail bases, called clubbing (with advanced disease) Tests that help diagnose IPF include the following:Bronchoscopy BronchoscopyBronchoscopy is a test to view the airways and diagnose lung disease. It may also be used during the treatment of some lung conditions.ImageRead Article Now Book Mark Article High resolution chest CT scan (HRCT) High resolution chest CT scan (HRCT)A chest CT (computed tomography) scan is an imaging method that uses x-rays to create cross-sectional pictures of the chest and upper abdomen....ImageRead Article Now Book Mark Article Chest x-ray Chest x-rayA chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.ImageRead Article Now Book Mark Article Echocardiogram EchocardiogramAn echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...ImageRead Article Now Book Mark Article Measurements of blood oxygen level (arterial blood gases) Pulmonary function tests Pulmonary function testsPulmonary function tests are a group of tests that measure breathing and how well the lungs are functioning.ImageRead Article Now Book Mark Article 6-minute walk test Tests for autoimmune diseases such as rheumatoid arthritis, lupus, or scleroderma Rheumatoid arthritisRheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...ImageRead Article Now Book Mark Article SclerodermaScleroderma is a disease that involves the buildup of scar-like tissue in the skin and elsewhere in the body. It also damages the cells that line th...ImageRead Article Now Book Mark Article Open lung (surgical) lung biopsy Treatment 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. Oxygen supportBecause of your medical problem, you may need to use oxygen to help you breathe. You will need to know how to use and store your oxygen.Read Article Now Book Mark Article 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.Home and lifestyle changesYou were in the hospital to treat your breathing problems that are caused by interstitial lung disease. This disease scars your lungs, which makes i...Read Article Now Book Mark Article A lung transplant may be considered for some people with advanced IPF.Lung transplantLung transplant is surgery to replace one or both diseased lungs with healthy lungs from a human donor.ImageRead Article Now Book Mark Article Support Groups 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/ Outlook (Prognosis) 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.Treatments that prolong lifeSometimes after injury or a long illness, the main organs of the body no longer work properly without support. Your health care provider may tell yo...Read Article Now Book Mark Article Advance care planningWhen you are very ill or injured, you may not be able to make health care choices for yourself. If you are unable to speak for yourself, your health...ImageRead Article Now Book Mark Article Possible Complications 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) Cor pulmonaleCor pulmonale is a condition that causes the right side of the heart to fail. Long-term high blood pressure in the arteries of the lung and right ve...ImageRead Article Now Book Mark Article Death When to Contact a Medical Professional 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 Open ReferencesReferencesNational 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.AllVideoImagesTogSpirometry - illustration Spirometry is a painless study of air volume and flow rate within the lungs. Spirometry is frequently used to evaluate lung function in people with obstructive or restrictive lung diseases such as asthma or cystic fibrosis.SpirometryillustrationClubbing - illustration Clubbing may result from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.ClubbingillustrationRespiratory system - illustration Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.Respiratory systemillustrationSpirometry - illustration Spirometry is a painless study of air volume and flow rate within the lungs. Spirometry is frequently used to evaluate lung function in people with obstructive or restrictive lung diseases such as asthma or cystic fibrosis.SpirometryillustrationClubbing - illustration Clubbing may result from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.ClubbingillustrationRespiratory system - illustration Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.Respiratory systemillustrationSelf Care Using oxygen at homeRelated Information Respiratory(Special Topic)Using oxygen at home(Self-Care) Review Date: 1/1/2020 Reviewed 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. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. 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