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Idiopathic Pulmonary Fibrosis (IPF): Treatments

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.

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(Source: NIH - National Cancer Institute)

Treatments for Idiopathic Pulmonary Fibrosis (IPF)

Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation (PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF).

Medicines

Currently, no medicines are proven to slow the progression of IPF.

Prednisone, azathioprine (A-zah-THI-o-preen), and N-acetylcysteine (a-SEH-til-SIS-tee-in) have been used to treat IPF, either alone or in combination. However, experts have not found enough evidence to support their use.

Prednisone

Prednisone is an anti-inflammatory medicine. You usually take it by mouth every day. However, your doctor may give it to you through a needle or tube inserted into a vein in your arm for several days. After that, you usually take it by mouth.

Because prednisone can cause serious side effects, your doctor may prescribe it for 3 to 6 months or less at first. Then, if it works for you, your doctor may reduce the dose over time and keep you on it longer....Read more about Idiopathic Pulmonary Fibrosis: Treatments
NIH - National Heart, Lung, and Blood Institute

What works? Research summarized

Evidence reviews

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

The study found that few interventions for idiopathic pulmonary fibrosis have any statistically significant effect, and identified a lack of studies on palliative care approaches. Few direct comparisons of treatments were identified; an indirect comparison was performed through network meta-analysis, but the results should be interpreted with caution.

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.

The effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: systematic review, network meta-analysis and health economic evaluation

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease with considerable impact on patients and carers as the disease progresses. Currently few treatments are available. We aimed to evaluate the clinical and cost-effectiveness of available treatments for IPF.

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Summaries for consumers

Benzodiazepines for the relief of breathlessness in advanced diseases in adults

Breathlessness is a common and distressing symptom in advanced cancer and other diseases at the end of life. Treating breathlessness sufficiently remains very difficult. Benzodiazepines are a group of sedating medicines (drugs), including lorazepam, clorazepate, diazepam, alprazolam, and temazepam, that are used mainly for sleep disturbance and anxiety, but are widely used for the relief of breathlessness.

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.
Lung Transplantation
A procedure in which diseased lungs are partially or totally replaced by lungs from a donor.
Lungs
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
Oxygen Therapy
Treatment in which a storage tank of oxygen or a machine called a compressor is used to give oxygen to people with breathing problems. The extra oxygen is breathed in along with normal air.
Pulmonary
Having to do with the lungs.

More about Idiopathic Pulmonary Fibrosis (IPF): Treatments

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Other terms to know: See all 10
Alveoli, Body Fiber, Bronchioles

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