Idiopathic pulmonary fibrosis

Common Name(s)

Idiopathic pulmonary fibrosis, Familial pulmonary fibrosis, Hamman-Rich Disease

Pulmonary fibrosis is the thickening and scarring of the lungs which impedes breathing. This makes it difficult for oxygen to pass from the lungs into the blood stream. It can be caused by variety of factors including airborne toxins, diseases, and even some medical treatments. Most of the time the cause of this scarring is unknown, which is referred to as idiopathic pulmonary fibrosis. Symptoms include shortness of breath, dry cough, fatigue, unexplained weight loss, and aching joints and muscles. Risk factors for pulmonary fibrosis include age, occupation, smoking, cancer treatments, and genetic factors. Complications that occur as a result of pulmonary fibrosis include pulmonary hypertension, right-sided heart failure, respiratory failure, and lung cancer. Although idiopathic pulmonary fibrosis usually only occurs in one person in a family, a small percentage of people with this disease have a least one other affected family member. When idiopathic pulmonary fibrosis occurs in more than one family member, it is referred to as familial pulmonary fibrosis.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Idiopathic 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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Dyskeratosis Congenita Outreach, Inc.

Our mission is to provide information and support services to families affected by Dyskeratosis Congenita, a Telomere Biology Disorder worldwide, to encourage the medical community's research in finding causes and effective treatments, and to facilitate improved diagnosis by educating medical providers.

Last Updated: 2 Oct 2014

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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 "Idiopathic 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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Dyskeratosis Congenita Outreach, Inc.

Our mission is to provide information and support services to families affected by Dyskeratosis Congenita, a Telomere Biology Disorder worldwide, to encourage the medical community's research in finding causes and effective treatments, and to facilitate improved diagnosis by educating medical providers.

http://www.dcoutreach.com

Last Updated: 2 Oct 2014

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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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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 "Idiopathic pulmonary fibrosis" returned 522 free, full-text research articles on human participants. First 3 results:

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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Integrated analyses identify the involvement of microRNA-26a in epithelial-mesenchymal transition during idiopathic pulmonary fibrosis.
 

Author(s): H Liang, Y Gu, T Li, Y Zhang, L Huangfu, M Hu, D Zhao, Y Chen, S Liu, Y Dong, X Li, Y Lu, B Yang, H Shan

Journal:

 

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, and highly lethal fibrotic lung disease with poor treatment and unknown etiology. Emerging evidence suggests that epithelial-mesenchymal transition (EMT) has an important role in repair and scar formation following epithelial ...

Last Updated: 23 May 2014

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A central role for G9a and EZH2 in the epigenetic silencing of cyclooxygenase-2 in idiopathic pulmonary fibrosis.
 

Author(s): William R Coward, Carol A Feghali-Bostwick, Gisli Jenkins, Alan J Knox, Linhua Pang

Journal: FASEB J.. 2014 Jul;28(7):3183-96.

 

Selective silencing of the cyclooxygenase-2 (COX-2) gene with the loss of the antifibrotic mediator prostaglandin E2 contributes to the fibrotic process in idiopathic pulmonary fibrosis (IPF). This study explored the role of G9a- and enhancer of zeste homolog 2 (EZH2)-mediated methylation ...

Last Updated: 29 Jul 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 "Idiopathic pulmonary fibrosis" returned 77 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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Idiopathic pulmonary fibrosis: from epithelial injury to biomarkers--insights from the bench side.
 

Author(s): Keren Borensztajn, Bruno Crestani, Martin Kolb

Journal: Respiration. 2013 ;86(6):441-52.

 

Idiopathic pulmonary fibrosis (IPF) is the most frequent fibrotic diffuse parenchymal lung disease. Its prognosis is devastating: >50% of the patients die within 3 years after diagnosis. Options for the treatment of IPF are limited and lung transplantation is the only 'curative' therapy. ...

Last Updated: 17 Jan 2014

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Advances in the study of biomarkers of idiopathic pulmonary fibrosis in Japan.
 

Author(s): Haibo Huang, Xiaonu Peng, Jun Nakajima

Journal: Biosci Trends. 2013 Aug;7(4):172-7.

 

Idiopathic pulmonary fibrosis is an intractable disease with a median survival time of 2 to 3 years. Serum levels of Krebs von den Lungen-6 (KL-6), surfactant protein A (SP-A), and surfactant protein D (SP-D) are useful biomarkers for idiopathic pulmonary fibrosis and they are widely ...

Last Updated: 23 Sep 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Pennsylvania Idiopathic Pulmonary Fibrosis Research Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 22 Jun 2012

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Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 28 Oct 2014

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Azathioprine and Prednisone in the Treatment of Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 16 Aug 2007

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