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 687 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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Comparisons of Prognosis between Surgically and Clinically Diagnosed Idiopathic Pulmonary Fibrosis Using Gap Model: A Korean National Cohort Study.
 

Author(s): Sang Hoon Lee, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Moo Suk Park

Journal: Medicine (Baltimore). 2016 Mar;95(11):e3105.

 

Although a multidisciplinary approach has become an important criterion for an idiopathic pulmonary fibrosis (IPF) diagnosis, lung biopsies remain crucial. However, the prognosis of patients with surgically diagnosed IPF (sIPF) is uncertain. We aimed to investigate the prognosis of ...

Last Updated: 18 Mar 2016

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Guidelines or guidance for better idiopathic pulmonary fibrosis management?
 

Author(s): Jürgen Behr

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a rare fibrotic interstitial lung disease with a relentlessly progressive course and fatal outcome. Guidelines summarizing the current evidence and providing evidence-based recommendations for the treatment of rare diseases such as IPF are important ...

Last Updated: 10 Feb 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 126 free, full-text review articles on human participants. First 3 results:

Update on diagnosis and treatment of idiopathic pulmonary fibrosis.
 

Author(s): José Baddini-Martinez, Bruno Guedes Baldi, Cláudia Henrique da Costa, Sérgio Jezler, Mariana Silva Lima, Rogério Rufino

Journal: J Bras Pneumol. ;41(5):454-66.

 

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent ...

Last Updated: 20 Nov 2015

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Novel mechanisms and treatment of idiopathic pulmonary fibrosis.
 

Author(s): Firas Elmufdi, Craig A Henke, David M Perlman, Rade Tomic, Hyun Joo Kim

Journal: Discov Med. 2015 Sep;20(109):145-53.

 

Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease of unknown etiology that carries a grim prognosis. Over the last few decades there have been significant advances in our understanding of the mechanisms that drive the fibrotic process. In this review, we discuss ...

Last Updated: 14 Oct 2015

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Epidemiological studies in idiopathic pulmonary fibrosis: pitfalls in methodologies and data interpretation.
 

Author(s): Antonella Caminati, Fabiana Madotto, Giancarlo Cesana, Sara Conti, Sergio Harari

Journal: Eur Respir Rev. 2015 Sep;24(137):436-44.

 

Data on incidence, prevalence and mortality of idiopathic pulmonary fibrosis (IPF) are sparse and vary across studies. The true incidence and prevalence of the disease are unknown. In general, the overall prevalence and incidence reported in European and Asian countries are lower ...

Last Updated: 1 Sep 2015

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Role of Genetics in Idiopathic Pulmonary Fibrosis (IPF)
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis; Familial Pulmonary Fibrosis; Idiopathic Interstitial Pneumonia; Familial Interstitial Pneumonia

 

Last Updated: 29 Mar 2016

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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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Pennsylvania Idiopathic Pulmonary Fibrosis Research Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 4 Jan 2016

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