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: 27 Nov 2012

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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: 27 Nov 2012

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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 1046 free, full-text research articles on human participants. First 3 results:

More on pulmonary fibrosis associated with aluminum trihydrate (Corian) dust.
 

Author(s): Ganesh Raghu, Daniel Xia, Jerrold L Abraham

Journal: N. Engl. J. Med.. 2014 Sep;371(10):973-4.

 

Last Updated: 4 Sep 2014

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Treatments for idiopathic pulmonary fibrosis.
 

Author(s): Luca Richeldi

Journal: N. Engl. J. Med.. 2014 Aug;371(8):783.

 

Last Updated: 21 Aug 2014

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Pulmonary fibrosis associated with aluminum trihydrate (Corian) dust.
 

Author(s): Paul Gannon, Robert W Rickard

Journal: N. Engl. J. Med.. 2014 May;370(22):2156-7.

 

Last Updated: 29 May 2014

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

Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials.
 

Author(s): Lesley Ann Saketkoo, Shikha Mittoo, Dörte Huscher, Dinesh Khanna, Paul F Dellaripa, Oliver Distler, Kevin R Flaherty, Sid Frankel, Chester V Oddis, Christopher P Denton, Aryeh Fischer, Otylia M Kowal-Bielecka, Daphne LeSage, Peter A Merkel, Kristine Phillips, David Pittrow, Jeffrey Swigris, Katerina Antoniou, Robert P Baughman, Flavia V Castelino, Romy B Christmann, Lisa Christopher-Stine, Harold R Collard, Vincent Cottin, Sonye Danoff, Kristin B Highland, Laura Hummers, Ami A Shah, Dong Soon Kim, David A Lynch, Frederick W Miller, Susanna M Proudman, Luca Richeldi, Jay H Ryu, Nora Sandorfi, Catherine Sarver, Athol U Wells, Vibeke Strand, Eric L Matteson, Kevin K Brown, James R Seibold,

Journal: Thorax. 2014 May;69(5):428-36.

 

Clinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may ...

Last Updated: 14 Apr 2014

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Fibrocytes in pulmonary fibrosis: a brief synopsis.
 

Author(s): Shyam Maharaj, Chiko Shimbori, Martin Kolb

Journal: Eur Respir Rev. 2013 Dec;22(130):552-7.

 

Fibrocytes are bone marrow-derived, circulating mesenchymal progenitor cells that play a role in several fibrotic disorders, including lung fibrosis. They are attracted to injured tissue by various chemokines. It is likely that fibrocytes play a detrimental role in tissue homeostasis ...

Last Updated: 2 Dec 2013

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Targeting platelet-derived growth factor as a therapeutic approach in pulmonary fibrosis.
 

Author(s): Yasuhiko Nishioka, Momoyo Azuma, Masami Kishi, Yoshinori Aono

Journal: J. Med. Invest.. 2013 ;60(3-4):175-83.

 

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disease characterized by the proliferation of fibroblasts and deposition of extracellular matrix. Since the prognosis of IPF is still poor, novel therapeutic modalities are strongly required. For this reason, to ...

Last Updated: 5 Nov 2013

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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: 31 Aug 2013

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Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 11 Sep 2014

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Supplemental Oxygen in Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 10 Oct 2013

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