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Idiopathic Pulmonary Fibrosis (IPF)

A disease in which the alveoli (tiny air sacs at the end of the bronchioles in the lungs) are overgrown with fibrous tissue. The cause of the disease is unknown and it gets worse over time. Symptoms include difficult, painful breathing and shortness of breath. NIH - National Cancer Institute

Idiopathic Pulmonary Fibrosis (IPF)

Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis.

As the lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (For more information, go to the "How the Lungs Work" section of this article.)

Sometimes doctors can find out what's causing fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF).

IPF is a serious disease that usually affects middle-aged and older adults. IPF varies from person to person. In some people, fibrosis happens quickly. In others, the process is much slower. In some people, the disease stays the same for years.

IPF has no cure yet. Many people live only about... Read more about Idiopathic Pulmonary Fibrosis

What works? Research summarized

Evidence reviews

[Efficacy of N-acetylcysteine for patients with idiopathic pulmonary fibrosis: a meta-analysis]

Bibliographic details: Fu XW, Tong X, Liu ST, Fan H.  [Efficacy of N-acetylcysteine for patients with idiopathic pulmonary fibrosis: a meta-analysis]. Chinese Journal of Evidence-Based Medicine 2014; 14(4): 449-455 Available from: http://dx.doi.org/10.7507/1672-2531.20140078

Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis: Idiopathic Pulmonary Fibrosis [Internet]

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease (ILD) of unknown origin. It is a difficult disease to diagnose and often requires the collaborative expertise of a chest physician, radiologist and histopathologist to reach a consensus diagnosis. Most people with idiopathic pulmonary fibrosis experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death. Specific pharmacological therapies for IPF are limited but the last decade has seen more trials of new drugs which have had a variable impact on clinical practice. A number of difficulties arise when undertaking clinical trials in IPF in terms of defining precise, diagnostic inclusion criteria and clinically meaningful end-points. However, such trials are the only way by which promising new treatments will come to benefit patients. Furthermore, it is only by performing rigorous clinical trials, we have learned that drugs once widely used to treat IPF may in fact have been harmful. The limitations of current pharmacological therapies for IPF highlight the importance of other forms of treatment including lung transplantation and best supportive care such as oxygen therapy, pulmonary rehabilitation and palliation of symptoms. These are interventions which justifiably require scrutiny in the context of healthcare delivery by the modern NHS. Despite the significant burden of disease caused by IPF, there is currently no established framework within the NHS for its diagnosis and management thus creating an environment in which significant variations in clinical care may occur. In recognition of this, the Department of Health commissioned the National Institute of Health and Care Excellence (NICE) to produce a guideline aimed at improving the care of people with IPF.

Interferon-gamma1b therapy in idiopathic pulmonary fibrosis: a metaanalysis

CONTEXT: Despite the investigation of multiple therapeutic options, idiopathic pulmonary fibrosis (IPF) remains a devastating, progressively fatal disease. Much interest has focused on the use of interferon (IFN)-gamma1b therapy, but the efficacy of this treatment has not been proven.

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Summaries for consumers

Currently there is no evidence to support the routine use of corticosteroids alone in the management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic disorder of unknown cause that is progressive and leads to death. Existing evidence about the treatment of IPF is difficult to interpret because of evolving diagnostic criteria and classification schemes, grouping together diverse entities with markedly different natural histories and responses to therapy. Therefore, because the old studies did not include only IPF patients, their results cannot be extended to IPF as they are currently defined. In addition, methodologic problems mainly related to the lack of randomized placebo‐controlled treatment trials have made evidence‐based conclusions difficult. A systematic search for high quality placebo‐controlled randomised trials comparing corticosteroid treatment with placebo in subjects with a diagnosis of IPF was unable to identify any studies suitable for inclusion in the review.

Non‐steroid agents for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a form of progressive lung disease which ultimately leads to death. The cause is unknown, but the disease is characterised by scar tissue in the lungs. This prevents the lungs from working effectively. Standard treatment uses oral corticosteroids in association with immunosuppressors, but there is uncertainty as to whether this treatment is effective. Immunosuppressive agents such as azathioprine and cyclophosphamide have been used to treat the disease because it is thought they might prevent inflammation. The review found 15 high quality trials of non‐steroid drugs tested in idiopathic pulmonary fibrosis patients. Notwithstanding the encouraging results of a first small study included in the first version of this review, the effects of interferon gamma‐1beta, as assessed by combining two subsequent large trials, were disappointing and failed to show an effect on improving survival. Four studies did evaluate pirfenidone, an anti‐fibrotic oral drug, on a large number of patients: although two of these studies have only been presented in conferences,combining the published and unpublished data showed a significant improvement of pirfenidone on progression‐free survival and a small increase in pulmonary function. Current evidence suggests a possible role for pirfenidone in the treatment of idiopathic pulmonary fibrosis, though data on survival are now needed. However, trials with other non‐steroid agents are currently ongoing and new evidence may become available soon.

Pulmonary fibrosis: What are the advantages or disadvantages of pirfenidone (Esbriet)?

Pirfenidone (trade name Esbriet) was approved in Germany at the beginning of 2011 for the treatment of mild to moderate idiopathic pulmonary fibrosis in adults.

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Terms to know

Alveoli
Tiny air sacs at the end of the bronchioles (tiny branches of air tubes) in the lungs. The alveoli are where the lungs and the bloodstream exchange carbon dioxide and oxygen. Carbon dioxide in the blood passes into the lungs through the alveoli. Oxygen in the lungs passes through the alveoli into the blood.
Body Fiber
In the body, fiber refers to tissue made of long threadlike cells, such as muscle fiber or nerve fiber.
Bronchioles
A tiny branch of air tubes in the lungs.
Fibrosis
Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue. Fibrosis is similar to the process of scarring.
Idiopathic
A medical term meaning "of unknown cause".
Interstitial
An interstitial space or interstice is an empty space or gap between spaces full of structure or matter.
Lungs
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
Pulmonary
Having to do with the lungs.
Respiratory Failure
Respiratory failure is a condition in which not enough oxygen passes from your lungs into your blood.

More about Idiopathic Pulmonary Fibrosis

Photo of an adult

Also called: Idiopathic fibrosing alveolitis (chronic form), Cryptogenic fibrosing alveolitis, Usual interstitial pneumonia, CFA

Other terms to know: See all 9
Alveoli, Body Fiber, Bronchioles

Related articles:
How the Lungs Work

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