Idiopathic pulmonary fibrosis

Common Name(s)

Idiopathic pulmonary fibrosis, Familial pulmonary fibrosis

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.

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: 1 Nov 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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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 "Idiopathic pulmonary fibrosis" for support, advocacy or research.

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: 1 Nov 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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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 "Idiopathic pulmonary fibrosis" returned 724 free, full-text research articles on human participants. First 3 results:

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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Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis.
 

Author(s): Michael Kreuter, Svenja Ehlers-Tenenbaum, Karin Palmowski, Jacques Bruhwyler, Ute Oltmanns, Thomas Muley, Claus Peter Heussel, Arne Warth, Martin Kolb, Felix J F Herth

Journal:

 

Comorbidities significantly influence the clinical course of idiopathic pulmonary fibrosis (IPF). However, their prognostic impact is not fully understood. We therefore aimed to determine the impact of comorbidities, as individual and as whole, on survival in IPF.

Last Updated: 30 Mar 2016

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Aberrant nonfibrotic parenchyma in idiopathic pulmonary fibrosis is correlated with decreased β-catenin inhibition and increased Wnt5a/b interaction.
 

Author(s): Kristina Rydell-Törmänen, Xiao-Hong Zhou, Oskar Hallgren, Jonas Einarsson, Leif Eriksson, Annika Andersson-Sjöland, Gunilla Westergren-Thorsson

Journal: Physiol Rep. 2016 Mar;4(5):.

 

Idiopathic pulmonary fibrosis (IPF), an insidious disease with grave prognosis, is characterized by heterogeneous fibrosis with densely fibrotic areas surrounded by nonfibrotic normal-looking tissue, believed to reflect a temporal development. The etiology is incompletely elucidated, ...

Last Updated: 21 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 "Idiopathic pulmonary fibrosis" returned 133 free, full-text review articles on human participants. First 3 results:

Role of matrix metalloproteinases in the pathogenesis of idiopathic pulmonary fibrosis.
 

Author(s): Annie Pardo, Sandra Cabrera, Mariel Maldonado, Moisés Selman

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a progressive and devastating lung disorder of unknown origin, with very poor prognosis and no effective treatment. The disease is characterized by abnormal activation of alveolar epithelial cells, which secrete numerous mediators involved in ...

Last Updated: 5 Mar 2016

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Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives.
 

Author(s): Michael Kreuter, Francesco Bonella, Marlies Wijsenbeek, Toby M Maher, Paolo Spagnolo

Journal: Biomed Res Int. 2015 ;2015():329481.

 

Idiopathic pulmonary fibrosis (IPF) is a devastating condition with a 5-year survival of approximately 20%. The disease primarily occurs in elderly patients. IPF is a highly heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of ...

Last Updated: 18 Jan 2016

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Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib.
 

Author(s): Francesco Bonella, Susanne Stowasser, Lutz Wollin

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and is characterized by a poor prognosis, with an estimated 5-year survival of approximately 20%. Progressive and irreversible lung functional impairment leads to chronic respiratory insufficiency ...

Last Updated: 30 Dec 2015

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Symptoms, Diagnosis, and Treatment

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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: 4 Jan 2016

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Pilot Trial Of Omeprazole in Idiopathic Pulmonary Fibrosis (IPF)
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 8 Oct 2015

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Evaluate the Safety and Efficacy of FG-3019 in Patients With Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 28 Jul 2016

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