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.

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: 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.

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

Lung fibroblasts from patients with idiopathic pulmonary fibrosis exhibit genome-wide differences in DNA methylation compared to fibroblasts from nonfibrotic lung.
 

Author(s): Steven K Huang, Anne M Scruggs, Richard C McEachin, Eric S White, Marc Peters-Golden

Journal:

 

Excessive fibroproliferation is a central hallmark of idiopathic pulmonary fibrosis (IPF), a chronic, progressive disorder that results in impaired gas exchange and respiratory failure. Fibroblasts are the key effector cells in IPF, and aberrant expression of multiple genes contributes ...

Last Updated: 13 Sep 2014

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The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis.
 

Author(s): Phillip L Molyneaux, Michael J Cox, Saffron A G Willis-Owen, Patrick Mallia, Kirsty E Russell, Anne-Marie Russell, Elissa Murphy, Sebastian L Johnston, David A Schwartz, Athol U Wells, William O C Cookson, Toby M Maher, Miriam F Moffatt

Journal: Am. J. Respir. Crit. Care Med.. 2014 Oct;190(8):906-13.

 

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease of unknown cause that leads to respiratory failure and death within 5 years of diagnosis. Overt respiratory infection and immunosuppression carry a high morbidity and mortality, and polymorphisms in genes related to ...

Last Updated: 16 Oct 2014

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

The psychometric properties of the St George's Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis: a literature review.
 

Author(s): Jeffrey J Swigris, Dirk Esser, Craig S Conoscenti, Kevin K Brown

Journal:

 

Assessment of health-related quality of life (HRQL) is particularly important in patients with progressive and incurable diseases such as idiopathic pulmonary fibrosis (IPF). The St George's Respiratory Questionnaire (SGRQ) has frequently been used to measure HRQL in patients with ...

Last Updated: 30 Aug 2014

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Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.
 

Author(s): Kristin D Kistler, Luba Nalysnyk, Philip Rotella, Dirk Esser

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung ...

Last Updated: 3 Sep 2014

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Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design?
 

Author(s): Steven D Nathan, Christopher S King

Journal:

 

Idiopathic pulmonary fibrosis (IPF) is a disease that carries a high mortality. Pulmonary hypertension (PH) frequently complicates the course of patients with IPF and is associated with significantly worse outcomes. Whether PH is a surrogate or driver of these worse outcomes remains ...

Last Updated: 25 Jul 2014

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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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

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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: 27 Jan 2015

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