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.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

Last Updated: 29 Apr 2014

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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.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

http://www.caringvoice.org

Last Updated: 29 Apr 2014

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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 1366 free, full-text research articles on human participants. First 3 results:

Targeting of the pulmonary capillary vascular niche promotes lung alveolar repair and ameliorates fibrosis.
 

Author(s): Zhongwei Cao, Raphael Lis, Michael Ginsberg, Deebly Chavez, Koji Shido, Sina Y Rabbany, Guo-Hua Fong, Thomas P Sakmar, Shahin Rafii, Bi-Sen Ding

Journal: Nat. Med.. 2016 Feb;22(2):154-62.

 

Although the lung can undergo self-repair after injury, fibrosis in chronically injured or diseased lungs can occur at the expense of regeneration. Here we study how a hematopoietic-vascular niche regulates alveolar repair and lung fibrosis. Using intratracheal injection of bleomycin ...

Last Updated: 5 Feb 2016

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Change in forced vital capacity and associated subsequent outcomes in patients with newly diagnosed idiopathic pulmonary fibrosis.
 

Author(s): William M Reichmann, Yanni F Yu, Dendy Macaulay, Eric Q Wu, Steven D Nathan

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a rare and serious disease characterized by progressive lung-function loss. Limited evidence has been published on the impact of lung-function loss on subsequent patient outcomes. This study examined change in forced vital capacity (FVC) across ...

Last Updated: 30 Dec 2015

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Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach.
 

Author(s): Spyros A Papiris, Konstantinos Kagouridis, Likurgos Kolilekas, Andriana I Papaioannou, Aneza Roussou, Christina Triantafillidou, Katerina Baou, Katerina Malagari, Stylianos Argentos, Anastasia Kotanidou, Anna Karakatsani, Effrosyni D Manali

Journal:

 

Idiopathic pulmonary fibrosis acute exacerbation (IPF-AE) constitutes IPF's most devastating event, representing the unexpected superimposition of diffuse alveolar damage of unknown etiology. Guidelines recommend high-dose steroids treatment despite unproven benefit. We hypothesized ...

Last Updated: 15 Dec 2015

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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 208 free, full-text review articles on human participants. First 3 results:

The Role of Mitochondrial DNA in Mediating Alveolar Epithelial Cell Apoptosis and Pulmonary Fibrosis.
 

Author(s): Seok-Jo Kim, Paul Cheresh, Renea P Jablonski, David B Williams, David W Kamp

Journal:

 

Convincing evidence has emerged demonstrating that impairment of mitochondrial function is critically important in regulating alveolar epithelial cell (AEC) programmed cell death (apoptosis) that may contribute to aging-related lung diseases, such as idiopathic pulmonary fibrosis ...

Last Updated: 15 Sep 2015

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Epidemiological studies in idiopathic pulmonary fibrosis: pitfalls in methodologies and data interpretation.
 

Author(s): Antonella Caminati, Fabiana Madotto, Giancarlo Cesana, Sara Conti, Sergio Harari

Journal: Eur Respir Rev. 2015 Sep;24(137):436-44.

 

Data on incidence, prevalence and mortality of idiopathic pulmonary fibrosis (IPF) are sparse and vary across studies. The true incidence and prevalence of the disease are unknown. In general, the overall prevalence and incidence reported in European and Asian countries are lower ...

Last Updated: 1 Sep 2015

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Idiopathic pulmonary fibrosis: from clinical trials to real-life experiences.
 

Author(s): Sergio Harari, Antonella Caminati

Journal: Eur Respir Rev. 2015 Sep;24(137):420-7.

 

Randomised controlled clinical trials are fundamental in medicine to develop new effective drugs and new therapeutic regimens and are the strength of evidence-based medicine. These studies allow us to avoid the repetition of misleading experiences that have been reported in the past, ...

Last Updated: 1 Sep 2015

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

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

Role of Genetics in Idiopathic Pulmonary Fibrosis (IPF)
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis; Familial Pulmonary Fibrosis; Idiopathic Interstitial Pneumonia; Familial Interstitial Pneumonia

 

Last Updated: 29 Mar 2016

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Pulmonary Fibrosis Contact Registry
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis of Any Cause; Primary Supporters/Caretakers of Patients With Pulmonary Fibrosis

 

Last Updated: 17 Nov 2014

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Genetics of the Combined Pulmonary Fibrosis and Emphysema Syndrome
 

Status: Recruiting

Condition Summary: Combined Pulmonary Fibrosis and Emphysema Syndrome; Pulmonary Fibrosis; Emphysema; Healthy Subjects

 

Last Updated: 7 May 2015

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