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 1606 free, full-text research articles on human participants. First 3 results:

Clinical impact of chronic obstructive pulmonary disease on non-cystic fibrosis bronchiectasis. A study on 1,790 patients from the Spanish Bronchiectasis Historical Registry.
 

Author(s): David De la Rosa, Miguel-Angel Martínez-Garcia, Rosa Maria Giron, Montserrat Vendrell, Casilda Olveira, Luis Borderias, Luis Maiz, Antoni Torres, Eva Martinez-Moragon, Olga Rajas, Francisco Casas, Rosa Cordovilla, Javier de Gracia

Journal:

 

Few studies have evaluated the coexistence of bronchiectasis (BE) and chronic obstructive pulmonary disease (COPD) in series of patients diagnosed primarily with BE. The aim of this study was to analyse the characteristics of patients with BE associated with COPD included in the Spanish ...

Last Updated: 25 May 2017

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Calcium-binding protein S100A4 confers mesenchymal progenitor cell fibrogenicity in idiopathic pulmonary fibrosis.
 

Author(s): Hong Xia, Adam Gilbertsen, Jeremy Herrera, Emilian Racila, Karen Smith, Mark Peterson, Timothy Griffin, Alexey Benyumov, Libang Yang, Peter B Bitterman, Craig A Henke

Journal: J. Clin. Invest.. 2017 Jun;127(7):2586-2597.

 

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a prevalence of 1 million persons worldwide. The fibrosis spreads from affected alveoli into contiguous alveoli and leads to death by asphyxiation. We previously discovered that the IPF lung harbors fibrogenic mesenchymal ...

Last Updated: 22 May 2017

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Effects of Feiwei granules in the treatment of idiopathic pulmonary fibrosis: a randomized and placebo-controlled trial.
 

Author(s): Yang Yu, Zengtao Sun, Liqing Shi, Yanping Zhang, Zhaoshan Zhou, Shunan Zhang, Enxiang Chao

Journal: J Tradit Chin Med. 2016 08;36(4):427-33.

 

To document the therapeutic effects of Feiwei granules (FGs) for idiopathic pulmonary fibrosis (IPF).

Last Updated: 1 May 2017

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

Impact of serum SP-A and SP-D levels on comparison and prognosis of idiopathic pulmonary fibrosis: A systematic review and meta-analysis.
 

Author(s): Kai Wang, Qing Ju, Jing Cao, Wenze Tang, Jian Zhang

Journal: Medicine (Baltimore). 2017 Jun;96(23):e7083.

 

Idiopathic pulmonary fibrosis (IPF) has a poor prognosis in general; however, it is heterogeneous to detect relative biomarkers for predicting the disease progression. Serum biomarkers can be conveniently collected to detect and help to differentially diagnose IPF and predict IPF ...

Last Updated: 7 Jun 2017

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Survival in Idiopathic Pulmonary Fibrosis: Perspectives from Pulmonary Arterial Hypertension.
 

Author(s): Toby M Maher, Fred Dejonckheere, Steven D Nathan

Journal: J Manag Care Spec Pharm. 2017 Mar;23(3-b Suppl):S3-S4.

 

Maher has received grants, consulting fees, and speaker fees from GlaxoSmithKline and UCB and grants from Novartis. He has also received consulting fees and speaker fees from AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Cipla, Lanthio, InterMune International AG (a wholly ...

Last Updated: 13 Mar 2017

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Systematic Review and Network Meta-analysis of Idiopathic Pulmonary Fibrosis Treatments.
 

Author(s): Kelly Fleetwood, Rachael McCool, Julie Glanville, Susan C Edwards, Sandro Gsteiger, Monica Daigl, Mark Fisher

Journal: J Manag Care Spec Pharm. 2017 Mar;23(3-b Suppl):S5-S16.

 

The antifibrotics pirfenidone and nintedanib are both approved for the treatment of idiopathic pulmonary fibrosis (IPF) by regulatory agencies and are recommended by health technology assessment bodies. Other treatments such as N-acetylcysteine are used in clinical practice but have ...

Last Updated: 13 Mar 2017

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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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Last Updated: 11 Oct 2015

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