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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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 1849 free, full-text research articles on human participants. First 3 results:

Mucosa-associated lymphoid tissue lymphoma of the trachea associated with idiopathic pulmonary fibrosis: A case report and literature review.
 

Author(s): June Hong Ahn, Jin Hong Chung, Kyeong-Cheol Shin, Eun Young Choi, Hyun Jung Jin, Joon Hyuk Choi, Kwan Ho Lee

Journal: Medicine (Baltimore). 2018 May;97(20):e10727.

 

Mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea is a rare disease that has been shown to be associated with chronic antigenic stimulation. There have been few reports of MALT lymphoma of the trachea in association with idiopathic pulmonary fibrosis (IPF).

Last Updated: 31 Dec 1969

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A case report of heterozygous TINF2 gene mutation associated with pulmonary fibrosis in a patient with dyskeratosis congenita.
 

Author(s): Hongchun Du, Yubiao Guo, Di Ma, Kejing Tang, Decheng Cai, Yifeng Luo, Canmao Xie

Journal: Medicine (Baltimore). 2018 May;97(19):e0724.

 

Dyskeratosis congenita (DC) is a rare inherited disease characterized by the classical mucocutaneous triad. Pulmonary fibrosis, bone marrow failure, and solid tumors are the main causes of mortality in DC. Pathogenic variants in TERT, TERC, and DKC1 have been identified in individuals ...

Last Updated: 31 Dec 1969

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It takes a village to care for the patient with idiopathic pulmonary fibrosis.
 

Author(s): Aryeh Fischer

Journal: Cleve Clin J Med. 2018 05;85(5):387-389.

 

Last Updated: 31 Dec 1969

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

Prognostic significance of pulmonary hypertension in patients with cystic fibrosis: A systematic review and meta-analysis.
 

Author(s): Diandian Li, Bo Wang, Hao Wang, Qun Liu

Journal: Medicine (Baltimore). 2018 Feb;97(7):e9708.

 

Pulmonary hypertension (PH) is frequently found in advanced parenchymal lung diseases like cystic fibrosis (CF), but the role played by PH in the clinical outcome of CF patients remains unclear. The aim of this study is to determine the influence of PH on survival in the CF population ...

Last Updated: 31 Dec 1969

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Acupuncture for idiopathic pulmonary fibrosis: Protocol for a systematic review.
 

Author(s): Yang Xie, Jia-Jia Wang, Gao-Yang Li, Xuan-Lin Li, Jian-Sheng Li

Journal: Medicine (Baltimore). 2017 Dec;96(52):e9114.

 

Patients with idiopathic pulmonary fibrosis (IPF) often suffer from progressive worsening of dyspnea and lung function. Although 2 drugs are now available for IPF treatment, many gaps are still to be filled. Evidences suggest that there may be some beneficial effect of acupuncture ...

Last Updated: 31 Dec 1969

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Role of MicroRNAs in TGF-β Signaling Pathway-Mediated Pulmonary Fibrosis.
 

Author(s): Hara Kang

Journal:

 

Pulmonary fibrosis is the most common form of interstitial lung disease. The transforming growth factor-β (TGF-β) signaling pathway is extensively involved in the development of pulmonary fibrosis by inducing cell differentiation, migration, invasion, or hyperplastic changes. Accumulating ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Assessment of Peripheral Endothelial Function in Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 11 Sep 2016

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Pilot Study of Pirfenidone in Pulmonary Fibrosis With Anti-myeloperoxydase Antibodies
 

Status: Recruiting

Condition Summary: Pulmonary Fibrosis

 

Last Updated: 16 Mar 2018

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Impact of a Systematic Palliative Care on Quality of Life, in Advanced Idiopathic Pulmonary Fibrosis.
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 24 Jan 2018

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