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What is Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis is a rare pulmonary disorder characterized by scarring of the lungs.

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Description

Idiopathic Pulmonary Fibrosis is a rare pulmonary disorder characterized by scarring of the lungs.

Acknowledgement

Acknowledgement of Idiopathic Pulmonary Fibrosis has not been added yet.

Prevalence

20.0http://ghr.nlm.nih.gov/condition/idiopathic-pulmonary-fibrosis

Synonyms

Synonyms for Idiopathic Pulmonary Fibrosis has not been added yet.

Cause

The underlying cause of IPF is unknown, which is why the condition is 'idiopathic’. Scientists believe that it is a combination of genetic and environmental factors (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). The damage that causes the severe symptoms takes place in the delicate epithelial cells that line the alveoli of the lungs. These are the millions of tiny, thin-walled air sacs found in the lungs, where exchange of oxygen and carbon dioxide occur between the respiratory and circulatory systems (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). A likely explanation is that the body attempts to repair damaged epithelial cells as a part of normal wound healing. However this process occurs abnormally, leading to progressive scarring and damage to the alveoli and surrounding lung tissue. The root cause of the disease is not known, but it is understood that genetic changes will increase a person's risk of developing IPF. Then, exposure to certain environmental factors acts as a “trigger” to cause the symptoms (http://ghr.nlm.nih.gov/condition/idiopathic-pulmonary-fibrosis). TERC and TERT are two mutated genes which have been found in about 15 percent of all IPF cases. Pulmonary fibrosis can also occur as a small part of another genetic disorder such as Hermansky-Pudlack syndrome. This disease is characterized by albinism, vision abnormalities and platelet dysfunction leading to prolonged bleeding (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/).

Symptoms

The early stages of IPF can present no symptoms at all (asymptomatic). The initial, characteristic symptom is shortness of breath during exertion such as exercise (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). Patients may also develop a dry, hacking cough that produces little to no sputum (phlegm, saliva etc.) (http://ghr.nlm.nih.gov/condition/idiopathic-pulmonary-fibrosis). Some additional symptoms include a loss of appetite leading to weight loss, and clubbing of the fingers and toes due to a lack of oxygen supply. If the disease continues to progress, severe symptoms such as respiratory failure, collapsed lungs (pneumothorax), and heart disease can occur (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). Patients suffering from IPF are also at a higher risk of developing lung cancer.

Diagnosis

The diagnosis of IPF can be done initially through identification of characteristic symptoms. If the disease is suspected, the diagnosis can then be confirmed by specialized tests including traditional chest x-rays (radiography), computer tomography (CT) scans, pulmonary function tests, blood tests, and the surgical removal and microscopic examination of lung tissue (lung biopsy) (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). The X-ray is the most common way of examining lung tissue for any damage. However, a special type of CT scanning known as high resolution computed tomography (HRCT) is often used for diagnosis because it produces detailed cross-sectional images of lung tissue structures. A doctor can also conduct pulmonary function tests, which measure how well the lungs exchange oxygen and how efficiently gases are transferred to and from the blood (http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/diagnosis). Finally, if a conclusive diagnosis cannot be made through the previously mentioned tests, a lung biopsy can be implemented. In this procedure, samples of lung tissue from several places within the lungs for inspection (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/).

Diagnostic Test

Diagnostic tests of Idiopathic Pulmonary Fibrosis has not been added yet

Treatment

The main goal of IPF treatment is to prevent more lung scarring, relieve symptoms, maintain the ability to be active, and improve the quality of life for the patient (https://rarediseases.info.nih.gov/gard/8609/idiopathic-pulmonary-fibrosis/resources/8). Recently in 2014, the Food and Drug Administration (FDA) approved two drugs for the treatment of the disease. The first, Ofev (nintedanib), is a kinase inhibitor that blocks multiple pathways that may be the cause of the scarring of lung tissue (http://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/). Esbriet (pirfenidone), another drug, also operates in a similar manner. Many IPF patients will undergo oxygen therapy at some point to increase oxygen levels in the bloodstream. In this treatment, supplemental oxygen is provided to the patient to compensate for the damaged lungs, and will reduce breathlessness and allow individuals to participate in physical activity (https://rarediseases.info.nih.gov/gard/8609/idiopathic-pulmonary-fibrosis/resources/8). If the patient does not respond well to other available treatments, a lung transplant may be required, provided that the patient is does not have any other medical conditions.

Prognosis

Unfortunately, the prognosis for most IPF patients is quite poor, since only 20-30% of affected people survive at least 5 years after diagnosis. Although the disease is variable in different patients, there are three main courses that could occur. IPF can be a slow progressive disease (the most common), a disease marked by episodes of acute exacerbations, or a rapidly progressive disease. The main factors that inhibit long-term survival include older age at diagnosis, extensive cigarette smoking, lower body mass index (BMI), more severe physiologic impairment, and the possible development of other complications (eg, pulmonary hypertension, emphysema, and bronchogenic cancer) (https://rarediseases.info.nih.gov/gard/8609/idiopathic-pulmonary-fibrosis/resources/15).

Tips or Suggestions

Tips or Suggestions of Idiopathic Pulmonary Fibrosis has not been added yet.

References

References of Idiopathic Pulmonary Fibrosis has not been added yet.

Upcoming podcast Created by icrispe
Last updated 10 Apr 2019, 02:21 AM

Posted by icrispe
10 Apr 2019, 02:21 AM

Hi everyone, I'm a volunteer with RareShare and Rare Genomics, and I'm hosting a podcast in a couple of weeks where I'll be interviewing a Pulmonary Fibrosis expert (Dr David Lederer), along with a patient. If anyone has any specific questions they would like me to ask the expert or the patient, please post them here by April 15.

Thanks so much,
Imogen

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Urge your congressperson to support PFREA HR 1027 Created by IPFdaughter
Last updated 23 Feb 2009, 12:24 AM

Posted by IPFdaughter
23 Feb 2009, 12:24 AM

The Pulmonary Fibrosis Research Enhancement Act (PFREA) has been re-introduced in Congress! This landmark bill, reintroduced on February 13th, establishes a national patient registry, calls for a national oversight program and efforts to increase public awareness of PF! Please note: the bill number has changed from the previous Congress. The NEW bill number is H.R. 1079. Tell your friends and family to call, email or write their congressman. Visit the Coalition for Pulminary Fibrosis website for an easy webform that will send an email for you (for free of course). http://www.coalitionforpf.org/cpf_join.php

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Community Podcasts

Title	Description	Date	Link
3 Perspectives on Facing a Rare Disease: A Patient, a Caregiver, and an Expert Discuss Diagnosis and Treatment of Pulmonary Fibrosis	Hear about rare disease diagnosis, treatment, and quality of life from the perspective of Pulmonary Fibrosis patient Jim Carns, his wife and caregiver Karen Carns, and Dr David Lederer, an expert from the Pulmonary Fibrosis Foundation who is also an Associate Professor at Columbia University. Jim tells us his diagnosis and treatment story, Karen explains what life is like as a caregiver or “care partner,” while Dr Lederer explains the causes of the disease, the treatment options, and upcoming Pulmonary Fibrosis research.	06/08/2019

Community External News Link

Title	Date	Link
Boehringer Ingelheim Gambles $2.62 Billion on Two Fibrotic Disease Deals	07/23/2019

Community Resources

Title	Description	Date	Link
Pulmonary Fibrosis Foundation	The Pulmonary Fibrosis Foundation is the primary organization dedicated to finding a cure for and raising awareness of Pulmonary Fibrosis.	03/20/2017

Clinical Trials

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

Complete the screening form.
Review the informed consent.
Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

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CurePF

I am a professional working with the Coalition for Pulmonary Fibrosis. I can assist people with questions about the disease and efforts to find treatments and a cure.