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

Suspected accelerated disease progression after discontinuation of nintedanib in patients with idiopathic pulmonary fibrosis: Two case reports.
 

Author(s): Satoshi Okamori, Takanori Asakura, Keita Masuzawa, Hiroyuki Yasuda, Hirofumi Kamata, Makoto Ishii, Tomoko Betsuyaku

Journal: Medicine (Baltimore). 2017 Dec;96(49):e9081.

 

The efficacy of nintedanib, a multitarget receptor tyrosine kinase inhibitor, has been demonstrated in recent randomized controlled trials involving patients with idiopathic pulmonary fibrosis (IPF). However, accelerated disease progression after nintedanib discontinuation has never been reported.

Last Updated: 31 Dec 1969

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Airway expression of Transient Receptor Potential (TRP) Vanniloid-1 and Ankyrin-1 channels is not increased in patients with Idiopathic Pulmonary Fibrosis.
 

Author(s): Nicola-Xan Hutchinson, Allen Gibbs, Amanda Tonks, Benjamin D Hope-Gill

Journal:

 

Dry cough is a common symptom described in patients with Idiopathic Pulmonary Fibrosis (IPF) and impairs quality of life. The exact mechanisms causing cough in IPF remain unclear, however evidence suggests altered cough neurophysiology and sensitisation plays a role; IPF patients ...

Last Updated: 31 Dec 1969

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Spermidine-mediated poly(lactic--glycolic acid) nanoparticles containing fluorofenidone for the treatment of idiopathic pulmonary fibrosis.
 

Author(s): Jing Tang, Jianming Li, Guo Li, Haitao Zhang, Ling Wang, Dai Li, Jinsong Ding

Journal:

 

Idiopathic pulmonary fibrosis is a progressive, fatal lung disease with poor survival. The advances made in deciphering this disease have led to the approval of different antifibrotic molecules, such as pirfenidone and nintedanib. An increasing number of studies with particles (liposomes, ...

Last Updated: 31 Dec 1969

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

Acupuncture for idiopathic pulmonary fibrosis: Protocol for a systematic review.
 

Author(s): Yang Xie, Jia-Jia Wang, Gao-Yang Li, Xuan-Lin Li, Jian-Sheng Li

Journal: Medicine (Baltimore). 2017 Dec;96(52):e9114.

 

Patients with idiopathic pulmonary fibrosis (IPF) often suffer from progressive worsening of dyspnea and lung function. Although 2 drugs are now available for IPF treatment, many gaps are still to be filled. Evidences suggest that there may be some beneficial effect of acupuncture ...

Last Updated: 31 Dec 1969

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Impact of serum SP-A and SP-D levels on comparison and prognosis of idiopathic pulmonary fibrosis: A systematic review and meta-analysis.
 

Author(s): Kai Wang, Qing Ju, Jing Cao, Wenze Tang, Jian Zhang

Journal: Medicine (Baltimore). 2017 Jun;96(23):e7083.

 

Idiopathic pulmonary fibrosis (IPF) has a poor prognosis in general; however, it is heterogeneous to detect relative biomarkers for predicting the disease progression. Serum biomarkers can be conveniently collected to detect and help to differentially diagnose IPF and predict IPF ...

Last Updated: 31 Dec 1969

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Survival in Idiopathic Pulmonary Fibrosis: Perspectives from Pulmonary Arterial Hypertension.
 

Author(s): Toby M Maher, Fred Dejonckheere, Steven D Nathan

Journal: J Manag Care Spec Pharm. 2017 Mar;23(3-b Suppl):S3-S4.

 

Maher has received grants, consulting fees, and speaker fees from GlaxoSmithKline and UCB and grants from Novartis. He has also received consulting fees and speaker fees from AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Cipla, Lanthio, InterMune International AG (a wholly ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Impact of a Systematic Palliative Care on Quality of Life, in Advanced Idiopathic Pulmonary Fibrosis.
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 24 Jan 2018

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Idiopathic Pulmonary Fibrosis (IPF) and the 3 Minutes Sit-to-stand Test
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 4 Sep 2017

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Using MRI to Visualize Regional Therapy Response in Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 10 Aug 2017

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