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

View Details
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

View Details
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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

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

View Details
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

View Details
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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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 1512 free, full-text research articles on human participants. First 3 results:

miR-30a as Potential Therapeutics by Targeting TET1 through Regulation of Drp-1 Promoter Hydroxymethylation in Idiopathic Pulmonary Fibrosis.
 

Author(s): Songzi Zhang, Huizhu Liu, Yuxia Liu, Jie Zhang, Hongbo Li, Weili Liu, Guohong Cao, Pan Xv, Jinjin Zhang, Changjun Lv, Xiaodong Song

Journal:

 

Several recent studies have indicated that miR-30a plays critical roles in various biological processes and diseases. However, the mechanism of miR-30a participation in idiopathic pulmonary fibrosis (IPF) regulation is ambiguous. Our previous study demonstrated that miR-30a may function ...

Last Updated: 15 Mar 2017

Go To URL
Pathway‑based detection of idiopathic pulmonary fibrosis at an early stage.
 

Author(s): Guojun Zhou, Fangxia Zhang, Yufang Liu, Bin Sun

Journal: Mol Med Rep. 2017 Apr;15(4):2023-2028.

 

Idiopathic pulmonary fibrosis (IPF) is the most common interstitial pneumonia and the most aggressive interstitial lung disease. Usually, IPF is confirmed by the histopathological pattern of typical interstitial pneumonia and requires an integrated multidisciplinary approach from ...

Last Updated: 5 Mar 2017

Go To URL
Genetic polymorphism in matrix metalloproteinase-9 and transforming growth factor-β1 and susceptibility to combined pulmonary fibrosis and emphysema in a Chinese population.
 

Author(s): Ling Xu, Wei Bian, Xiao-Hua Gu, Ce Shen

Journal: Kaohsiung J. Med. Sci.. 2017 Mar;33(3):124-129.

 

In this study, we aimed to explore the association of genetic polymorphism in matrix metalloproteinase-9 (MMP-9) and transforming growth factor-β1 (TGF-β1) and the susceptibility to combined pulmonary fibrosis and emphysema (CPFE). We examined the polymorphisms of the MMP-9 C-1562T ...

Last Updated: 3 Mar 2017

Go To URL

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

Diabetic Microvascular Disease and Pulmonary Fibrosis: The Contribution of Platelets and Systemic Inflammation.
 

Author(s): Rekha Jagadapillai, Madhavi J Rane, Xingyu Lin, Andrew M Roberts, Gary W Hoyle, Lu Cai, Evelyne Gozal

Journal:

 

Diabetes is strongly associated with systemic inflammation and oxidative stress, but its effect on pulmonary vascular disease and lung function has often been disregarded. Several studies identified restrictive lung disease and fibrotic changes in diabetic patients and in animal models ...

Last Updated: 11 Nov 2016

Go To URL
Cough in idiopathic pulmonary fibrosis.
 

Author(s): Mirjam J G van Manen, Surinder S Birring, Carlo Vancheri, Vincent Cottin, Elisabetta A Renzoni, Anne-Marie Russell, Marlies S Wijsenbeek

Journal: Eur Respir Rev. 2016 Sep;25(141):278-86.

 

Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in ...

Last Updated: 1 Sep 2016

Go To URL
Efficacy of N-Acetylcysteine in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
 

Author(s): Tong Sun, Jing Liu, De Wei Zhao

Journal: Medicine (Baltimore). 2016 May;95(19):e3629.

 

There are a number of conflicting reports describing the clinical outcomes of using N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis. We have, therefore, performed a meta-analysis to evaluate the efficacy of N-acetylcysteine, compared with control, for the treatment ...

Last Updated: 14 May 2016

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
Top

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

Go to URL
Assessment of Peripheral Endothelial Function in Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 11 Sep 2016

Go to URL

Last Updated: 11 Oct 2015

Go to URL