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 1103 free, full-text research articles on human participants. First 3 results:

Mixed connective tissue disease associated with autoimmune hepatitis and pulmonary fibrosis.
 

Author(s): Eva Zold, Edit Bodolay, Balazs Dezső, Györgyike Soos, Britt Nakken, Peter Szodoray

Journal: Isr. Med. Assoc. J.. 2014 Nov;16(11):733-4.

 

Last Updated: 6 Jan 2015

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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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Blockade of IL-6 Trans signaling attenuates pulmonary fibrosis.
 

Author(s): Thanh-Thuy T Le, Harry Karmouty-Quintana, Ernestina Melicoff, Thanh-Truc T Le, Tingting Weng, Ning-Yuan Chen, Mesias Pedroza, Yang Zhou, Jonathan Davies, Kemly Philip, Jose Molina, Fayong Luo, Anuh T George, Luis J Garcia-Morales, Raquel R Bunge, Brian A Bruckner, Matthias Loebe, Harish Seethamraju, Sandeep K Agarwal, Michael R Blackburn

Journal: J. Immunol.. 2014 Oct;193(7):3755-68.

 

Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease with progressive fibrosis and death within 2-3 y of diagnosis. IPF incidence and prevalence rates are increasing annually with few effective treatments available. Inhibition of IL-6 results in the attenuation of pulmonary ...

Last Updated: 20 Sep 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 158 free, full-text review articles on human participants. First 3 results:

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition.
 

Author(s): Olívia Meira Dias, Bruno Guedes Baldi, André Nathan Costa, Carlos Roberto Ribeiro Carvalho

Journal: J Bras Pneumol. ;40(3):304-12.

 

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe ...

Last Updated: 17 Jul 2014

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Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models.
 

Author(s): Wim A Wuyts, Fedro A Peccatori, Anne-Marie Russell

Journal: Eur Respir Rev. 2014 Jun;23(132):231-8.

 

The progressive and highly variable course of idiopathic pulmonary fibrosis (IPF) can present patients and their families with various challenges at different points of the disease. Structured communication between the healthcare professional and the patient is vital to ensure the ...

Last Updated: 2 Jun 2014

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Staging of idiopathic pulmonary fibrosis: past, present and future.
 

Author(s): Martin Kolb, Harold R Collard

Journal: Eur Respir Rev. 2014 Jun;23(132):220-4.

 

Idiopathic pulmonary fibrosis (IPF) is traditionally staged with terms such as "mild", "severe", "early" and "advanced" based on pulmonary function tests. This approach allows physicians to monitor disease progression and advise patients and their families. However, it is not known ...

Last Updated: 2 Jun 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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Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 27 Jan 2015

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Supplemental Oxygen in Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 10 Oct 2013

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