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

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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Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF).
 

Author(s): Noriyuki Enomoto, Hideki Kusagaya, Yoshiyuki Oyama, Masato Kono, Yusuke Kaida, Shigeki Kuroishi, Dai Hashimoto, Tomoyuki Fujisawa, Koshi Yokomura, Naoki Inui, Yutaro Nakamura, Takafumi Suda

Journal:

 

The pathological appearance of idiopathic pleuroparenchymal fibroelastosis (IPPFE) with hematoxylin-eosin staining is similar to that of usual interstitial pneumonia (UIP) in patients with idiopathic pulmonary fibrosis (IPF). The amount of elastic fibers (EF) and detailed differences ...

Last Updated: 4 Jun 2014

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Management of idiopathic pulmonary fibrosis: selected case reports.
 

Author(s): Michael Kreuter, Peter Kardos, Victor Hoffstein

Journal: Eur Respir Rev. 2014 Jun;23(132):239-48.

 

In 2011, revised international guidelines were issued jointly by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Latin American Thoracic Association, which provide a valuable framework for the diagnosis and management of idiopathic ...

Last Updated: 2 Jun 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 86 free, full-text review articles on human participants. First 3 results:

Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models.
 

Author(s): Wim A Wuyts, Fedro A Peccatori, Anne-Marie Russell

Journal: Eur Respir Rev. 2014 Jun;23(132):231-8.

 

The progressive and highly variable course of idiopathic pulmonary fibrosis (IPF) can present patients and their families with various challenges at different points of the disease. Structured communication between the healthcare professional and the patient is vital to ensure the ...

Last Updated: 2 Jun 2014

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Staging of idiopathic pulmonary fibrosis: past, present and future.
 

Author(s): Martin Kolb, Harold R Collard

Journal: Eur Respir Rev. 2014 Jun;23(132):220-4.

 

Idiopathic pulmonary fibrosis (IPF) is traditionally staged with terms such as "mild", "severe", "early" and "advanced" based on pulmonary function tests. This approach allows physicians to monitor disease progression and advise patients and their families. However, it is not known ...

Last Updated: 2 Jun 2014

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Imaging: how to recognise idiopathic pulmonary fibrosis.
 

Author(s): Anand Devaraj

Journal: Eur Respir Rev. 2014 Jun;23(132):215-9.

 

It is well known that high-resolution computed tomography (HRCT) is an essential component of the diagnostic pathway in idiopathic pulmonary fibrosis (IPF). Honeycombing, a common feature of IPF seen on HRCT, is crucial for an accurate diagnosis. Unfortunately, identification of honeycombing ...

Last Updated: 2 Jun 2014

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Symptoms, Diagnosis, and Treatment

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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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