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

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: 27 Nov 2012

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 "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

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: 27 Nov 2012

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 "Idiopathic pulmonary fibrosis" returned 496 free, full-text research articles on human participants. First 3 results:

Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study.
 

Author(s): Imre Noth, Yingze Zhang, Shwu-Fan Ma, Carlos Flores, Mathew Barber, Yong Huang, Steven M Broderick, Michael S Wade, Pirro Hysi, Joseph Scuirba, Thomas J Richards, Brenda M Juan-Guardela, Rekha Vij, Meilan K Han, Fernando J Martinez, Karl Kossen, Scott D Seiwert, Jason D Christie, Dan Nicolae, Naftali Kaminski, Joe G N Garcia

Journal: Lancet Respir Med. 2013 Jun;1(4):309-17.

 

Idiopathic pulmonary fibrosis (IPF) is a devastating disease that probably involves several genetic loci. Several rare genetic variants and one common single nucleotide polymorphism (SNP) of MUC5B have been associated with the disease. Our aim was to identify additional common variants ...

Last Updated: 16 Jan 2014

Go To URL
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

Go To URL
Screening and further analyzing differentially expressed genes in acute idiopathic pulmonary fibrosis with DNA microarray.
 

Author(s): F Min, F Gao, Z Liu

Journal: Eur Rev Med Pharmacol Sci. 2013 Oct;17(20):2784-90.

 

Acute idiopathic pulmonary fibrosis (IPF) is a serious and progressive form of lung disease, and millions of people suffer from this disease in the world. To provide clues for getting a better understanding of the mechanism of this disease, we identified and further analyzed the differential ...

Last Updated: 31 Oct 2013

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 "Idiopathic pulmonary fibrosis" returned 74 free, full-text review articles on human participants. First 3 results:

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

Go To URL
The role of infection in the pathogenesis of idiopathic pulmonary fibrosis.
 

Author(s): Philip L Molyneaux, Toby M Maher

Journal: Eur Respir Rev. 2013 Sep;22(129):376-81.

 

Idiopathic pulmonary fibrosis (IPF) is a progressive, and invariably fatal, condition that is believed to arise in genetically susceptible individuals as a consequence of an aberrant wound-healing response following repetitive alveolar injury. The exact triggers, which initiate the ...

Last Updated: 2 Sep 2013

Go To URL
Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future.
 

Author(s): Katerina M Antoniou, George A Margaritopoulos, Nikos M Siafakas

Journal: Eur Respir Rev. 2013 Sep;22(129):281-91.

 

During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, ...

Last Updated: 2 Sep 2013

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

Azathioprine and Prednisone in the Treatment of Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 16 Aug 2007

Go to URL
Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Not yet recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 3 Mar 2014

Go to URL
The Effect of Pirfenidone on Cough in Patients With Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 8 Dec 2013

Go to URL