Pulmonary Fibrosis

Common Name(s)

Pulmonary Fibrosis, Pulmonary fibrosis /granuloma

Pulmonary fibrosis, meaning scarring in the lung tissue, is an umbrella term for more than 200 different lung diseases that all look very much alike. In pulmonary fibrosis, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising. The most common symptoms of pulmonary fibrosis are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. This can typically be managed with medications to relax the lung tissue and limit the development of more scar tissue. However, if the disease progresses, lung transplant may be necessary.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pulmonary Fibrosis" for support, advocacy or research.

Coalition for Pulmonary Fibrosis

The Coalition for Pulmonary Fibrosis (CPF) is a 501(c)(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (IPF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease.

Last Updated: 28 Apr 2014

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International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

Last Updated: 18 Apr 2010

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Pulmonary Fibrosis Foundation

The mission of the Pulmonary Fibrosis Foundation (PFF) is to help find a cure for idiopathic pulmonary fibrosis (IPF), advocate for the pulmonary fibrosis community, promote disease awareness, and provide a compassionate environment for patients and their families.

Last Updated: 5 Jun 2015

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pulmonary Fibrosis" for support, advocacy or research.

Coalition for Pulmonary Fibrosis

The Coalition for Pulmonary Fibrosis (CPF) is a 501(c)(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (IPF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease.

http://www.coalitionforpf.org

Last Updated: 28 Apr 2014

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International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

http://www.sclero.org/

Last Updated: 18 Apr 2010

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Pulmonary Fibrosis Foundation

The mission of the Pulmonary Fibrosis Foundation (PFF) is to help find a cure for idiopathic pulmonary fibrosis (IPF), advocate for the pulmonary fibrosis community, promote disease awareness, and provide a compassionate environment for patients and their families.

http://www.pulmonaryfibrosis.org

Last Updated: 5 Jun 2015

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Pulmonary Fibrosis" returned 1497 free, full-text research articles on human participants. First 3 results:

Plasma microRNAs are associated with acute exacerbation in idiopathic pulmonary fibrosis.
 

Author(s): Haiyan Min, Shanshan Fan, Shiyu Song, Yi Zhuang, Hui Li, Yongzheng Wu, Hourong Cai, Long Yi, Jinghong Dai, Qian Gao

Journal:

 

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has high short-term mortality with unknown causes. To predict this malignant condition in clinics is challenging. In this study, we aim to demonstrate whether there are miRNAs that differ between AE-IPF and stable IPF, which ...

Last Updated: 24 Nov 2016

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Association between pulmonary fibrosis and osteoporosis in the elderly people: A case-control study.
 

Author(s): Zhong Xie, Yanhong He, Yongqiang Sun, Zhanzhan Lin, Mingzhi Yang, Qian Liu, Sihai Liu

Journal: Medicine (Baltimore). 2016 Nov;95(44):e5239.

 

In this case-control study, we evaluated the association between osteoporosis and pulmonary fibrosis in the elderly. Participants were divided into a test group and a control group depending on bone mineral density and grid pattern changes of pulmonary fibrosis. We measured general ...

Last Updated: 18 Nov 2016

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Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.
 

Author(s): Muhammad Danasabe Isah, Aminu Abbas, Abdullahi A Abba, Mohammed Umar

Journal: Ann Afr Med. ;15(4):204-206.

 

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. ...

Last Updated: 17 Nov 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Pulmonary Fibrosis" returned 222 free, full-text review articles on human participants. First 3 results:

Efficacy of N-Acetylcysteine in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
 

Author(s): Tong Sun, Jing Liu, De Wei Zhao

Journal: Medicine (Baltimore). 2016 May;95(19):e3629.

 

There are a number of conflicting reports describing the clinical outcomes of using N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis. We have, therefore, performed a meta-analysis to evaluate the efficacy of N-acetylcysteine, compared with control, for the treatment ...

Last Updated: 14 May 2016

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Aging and Pulmonary Fibrosis.
 

Author(s): Moisés Selman, Ivette Buendía-Roldán, Annie Pardo

Journal: Rev. Invest. Clin.. ;68(2):75-83.

 

Idiopathic pulmonary fibrosis is a chronic, progressive, and usually fatal lung disorder of unknown etiology. The disease likely results from the interaction of genetic susceptibility architecture, environmental factors such as smoking, and an abnormal epigenetic reprogramming that ...

Last Updated: 22 Apr 2016

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[Current management of idiopathic pulmonary fibrosis].
 

Author(s): Zehra Yaşar, Erdoğan Çetinkaya

Journal: Tuberk Toraks. 2015 ;63(4):278-90.

 

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and eventually fatal chronic interstitial pneumonia limited to the lung and associated with the histological and/or radiological pattern of usual interstitial pneumonia.The mean age of the disease is 65 and it is more ...

Last Updated: 11 Mar 2016

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Explanted Lung Tissues With Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 19 Sep 2016

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Assessment of Peripheral Endothelial Function in Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 11 Sep 2016

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Supplemental Oxygen in Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 14 Apr 2015

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