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 1417 free, full-text research articles on human participants. First 3 results:

Biomarkers in Exhaled Breath Condensate Are Not Predictive for Pulmonary Exacerbations in Children with Cystic Fibrosis: Results of a One-Year Observational Study.
 

Author(s): Marieke van Horck, Ariel Alonso, Geertjan Wesseling, Karin de Winter-de Groot, Wim van Aalderen, Han Hendriks, Bjorn Winkens, Ger Rijkers, Quirijn Jöbsis, Edward Dompeling

Journal:

 

Cystic Fibrosis (CF) is characterized by chronically inflamed airways, and inflammation even increases during pulmonary exacerbations. These adverse events have an important influence on the well-being, quality of life, and lung function of patients with CF. Prediction of exacerbations ...

Last Updated: 7 Apr 2016

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Normal Lung Quantification in Usual Interstitial Pneumonia Pattern: The Impact of Threshold-based Volumetric CT Analysis for the Staging of Idiopathic Pulmonary Fibrosis.
 

Author(s): Hirotsugu Ohkubo, Yoshihiro Kanemitsu, Takehiro Uemura, Osamu Takakuwa, Masaya Takemura, Ken Maeno, Yutaka Ito, Tetsuya Oguri, Nobutaka Kazawa, Ryuji Mikami, Akio Niimi

Journal:

 

Although several computer-aided computed tomography (CT) analysis methods have been reported to objectively assess the disease severity and progression of idiopathic pulmonary fibrosis (IPF), it is unclear which method is most practical. A universal severity classification system ...

Last Updated: 1 Apr 2016

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Comparisons of Prognosis between Surgically and Clinically Diagnosed Idiopathic Pulmonary Fibrosis Using Gap Model: A Korean National Cohort Study.
 

Author(s): Sang Hoon Lee, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Moo Suk Park

Journal: Medicine (Baltimore). 2016 Mar;95(11):e3105.

 

Although a multidisciplinary approach has become an important criterion for an idiopathic pulmonary fibrosis (IPF) diagnosis, lung biopsies remain crucial. However, the prognosis of patients with surgically diagnosed IPF (sIPF) is uncertain. We aimed to investigate the prognosis of ...

Last Updated: 18 Mar 2016

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

Update on diagnosis and treatment of idiopathic pulmonary fibrosis.
 

Author(s): José Baddini-Martinez, Bruno Guedes Baldi, Cláudia Henrique da Costa, Sérgio Jezler, Mariana Silva Lima, Rogério Rufino

Journal: J Bras Pneumol. ;41(5):454-66.

 

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent ...

Last Updated: 20 Nov 2015

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Novel mechanisms and treatment of idiopathic pulmonary fibrosis.
 

Author(s): Firas Elmufdi, Craig A Henke, David M Perlman, Rade Tomic, Hyun Joo Kim

Journal: Discov Med. 2015 Sep;20(109):145-53.

 

Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease of unknown etiology that carries a grim prognosis. Over the last few decades there have been significant advances in our understanding of the mechanisms that drive the fibrotic process. In this review, we discuss ...

Last Updated: 14 Oct 2015

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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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Symptoms, Diagnosis, and Treatment

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

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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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