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 1157 free, full-text research articles on human participants. First 3 results:

Two therapies now available for idiopathic pulmonary fibrosis.
 

Author(s): Thomas Morrow

Journal: Manag Care. 2014 Dec;23(12):47-8.

 

Last Updated: 5 May 2015

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Gene expression in transformed lymphocytes reveals variation in endomembrane and HLA pathways modifying cystic fibrosis pulmonary phenotypes.
 

Author(s): Wanda K O'Neal, Paul Gallins, Rhonda G Pace, Hong Dang, Whitney E Wolf, Lisa C Jones, XueLiang Guo, Yi-Hui Zhou, Vered Madar, Jinyan Huang, Liming Liang, Miriam F Moffatt, Garry R Cutting, Mitchell L Drumm, Johanna M Rommens, Lisa J Strug, Wei Sun, Jaclyn R Stonebraker, Fred A Wright, Michael R Knowles

Journal: Am. J. Hum. Genet.. 2015 Feb;96(2):318-28.

 

Variation in cystic fibrosis (CF) phenotypes, including lung disease severity, age of onset of persistent Pseudomonas aeruginosa (P. aeruginosa) lung infection, and presence of meconium ileus (MI), has been partially explained by genome-wide association studies (GWASs). It is not ...

Last Updated: 9 Feb 2015

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Blocking follistatin-like 1 attenuates bleomycin-induced pulmonary fibrosis in mice.
 

Author(s): Yingying Dong, Yan Geng, Lian Li, Xiaohe Li, Xiaohua Yan, Yinshan Fang, Xinxin Li, Siyuan Dong, Xue Liu, Xue Li, Xiuhong Yang, Xiaohong Zheng, Ting Xie, Jiurong Liang, Huaping Dai, Xinqi Liu, Zhinan Yin, Paul W Noble, Dianhua Jiang, Wen Ning

Journal: J. Exp. Med.. 2015 Feb;212(2):235-52.

 

Progressive tissue fibrosis is a cause of major morbidity and mortality. Pulmonary fibrosis is an epithelial-mesenchymal disorder in which TGF-β1 plays a central role in pathogenesis. Here we show that follistatin-like 1 (FSTL1) differentially regulates TGF-β and bone morphogenetic ...

Last Updated: 10 Feb 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 171 free, full-text review articles on human participants. First 3 results:

The effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: systematic review, network meta-analysis and health economic evaluation.
 

Author(s): Emma Loveman, Vicky R Copley, Jill L Colquitt, David A Scott, Andy J Clegg, Jeremy Jones, Katherine M A O'Reilly, Sally Singh, Claudia Bausewein, Athol Wells

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease with considerable impact on patients and carers as the disease progresses. Currently few treatments are available. We aimed to evaluate the clinical and cost-effectiveness of available treatments for IPF.

Last Updated: 27 Nov 2014

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The psychometric properties of the St George's Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis: a literature review.
 

Author(s): Jeffrey J Swigris, Dirk Esser, Craig S Conoscenti, Kevin K Brown

Journal:

 

Assessment of health-related quality of life (HRQL) is particularly important in patients with progressive and incurable diseases such as idiopathic pulmonary fibrosis (IPF). The St George's Respiratory Questionnaire (SGRQ) has frequently been used to measure HRQL in patients with ...

Last Updated: 30 Aug 2014

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Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.
 

Author(s): Kristin D Kistler, Luba Nalysnyk, Philip Rotella, Dirk Esser

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung ...

Last Updated: 3 Sep 2014

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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

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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Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 3 Jun 2015

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