Idiopathic pulmonary fibrosis (IPF) definition and facts
Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive
fibrosing interstitial lung disease that can cause pneumonia.
- Idiopathic pulmonary fibrosis has no known cause.
- Symptoms of idiopathic pulmonary fibrosis include a six-month history of
- Stages of idiopathic pulmonary fibrosis are not standardized, but some staging systems are designed to
include certain criteria: traditionally, idiopathic pulmonary fibrosis has been staged as mild, moderate,
- Doctors who treat idiopathic pulmonary fibrosis are primary care physicians, pulmonologists,
gastroenterologists, and transplant surgeons.
- The diagnosis of idiopathic pulmonary fibrosis is one of exclusion of other interstitial lung diseases.
In addition, high-resolution computed tomography (CT scans) and surgical biopsy of the lung
tissue help to confirm the diagnosis.
- Treatment of idiopathic pulmonary fibrosis may involve medications, supportive therapy, and/or lung
- Guidelines from many organizations recommend the use of
- Lung transplantation is the treatment option that does show survival benefit
over medical therapy.
- Because idiopathic pulmonary fibrosis is idiopathic (no known cause), researchers are not sure how to
prevent the disease, but suggest that avoiding smoking and reducing or
eliminating exposure to toxins or fumes that may damage the lungs may decrease
risk of idiopathic pulmonary fibrosis.
- Life expectancy is about 2 to 5 years. Patients who undergo lung
transplant have a mortality rate of about 50% to 56% after five years so the
prognosis of idiopathic pulmonary fibrosis is usually fair to poor. Patients and
their families are urged to contact support groups included in this
What is idiopathic pulmonary fibrosis (IPF)?
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of
progressive fibrosis causes interstitial pneumonia, usually in older adults and
is limited to the lungs. Fibrotic (scar-like) tissue develops in the airways of
the lungs, making it difficult for the body to get the oxygen it needs.
Idiopathic pulmonary fibrosis differs from pulmonary fibrosis in that the cause
is unknown whereas there are known causes of pulmonary fibrosis.
This article will discuss idiopathic pulmonary fibrosis. Pulmonary fibrosis is the subject of another
article. Though not very common, idiopathic pulmonary fibrosis is considered to be a debilitating,
incurable, and terminal disease by many health-care professionals and
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Pulmonary Fibrosis Symptoms and Signs
Symptoms of pulmonary fibrosis vary depending on the cause of the disease.
The severity of symptoms and the progression (worsening) of symptoms over time
can vary, and may include:
- Shortness of breath (in rare cases for weeks to months from the onset of
- Chronic wheezing
- Abnormal, rapid breathing
- Chest pain
- Diminished exercise tolerance
- Digital clubbing
What are the signs and symptoms of idiopathic pulmonary fibrosis (IPF)?
The symptoms of idiopathic pulmonary fibrosis are nonspecific, and are similar to many other types of
pulmonary cardiac diseases. The majority of people with the disease have about a 6
month or more history of shortness of breath and/or nonproductive cough. About
5% of individuals with IPF have no symptoms, but are diagnosed when being examined for other
medical problems. The signs and symptoms that may occur in some people with IPF are:
- Six months or greater history of
shortness of breath and/or cough
- Mild fevers
- Weight loss
- Joint discomfort
- Muscle pain
- Clubbing or enlargement of fingers
- Ankle swelling
- Heart murmur (tricuspid regurgitation)
What causes idiopathic pulmonary fibrosis (IPF)?
Inclusion of the word "idiopathic" means that this type of fibrosis has an
unknown cause. Current hypotheses include exposure to some type of agent (for
example, environmental dust, viral infection, reflux, aspiration of
gastrointestinal contents, and others) that leads to the alveolar damage in the
lungs. Researchers further hypothesize that in attempt to repair the alveolar
damage, fibrosis and lung scarring occur to produce eventually clinical
What are the stages of idiopathic pulmonary fibrosis (IPF)?
Staging systems for idiopathic pulmonary fibrosis may help health-care
professionals and researchers to better define
the medical condition of their patients. However, for idiopathic pulmonary
fibrosis, staging systems are
constantly being redefined. For example, the first staging systems traditionally
considered the disease as mild, moderate, or severe; while others considered
stages as early or advanced. The stages are relatively arbitrary and loosely
based on pulmonary function tests. Unfortunately, there are no standardized
definitions for mild, moderate, and severe idiopathic pulmonary fibrosis. Though arbitrary, these
staging systems give health-care professionals a framework to diagnose and treat patients with
idiopathic pulmonary fibrosis.
For example, some doctors consider treatment with pirfenidone for mild to
moderate disease, and lung transplantation for severe idiopathic pulmonary
fibrosis. There are researchers
that have developed novel idiopathic pulmonary fibrosis staging systems based on other criteria such as
age, hospitalization, baseline lung function, and a 24-week change in function,
while other researchers choose other parameters such as various physiological
lung measurements. The patient and their physician(s) can discuss which staging
system they choose to utilize.
Which specialties of doctors treat idiopathic pulmonary fibrosis (IPF)?
Although primary care physicians help manage patients with idiopathic
pulmonary fibrosis, usually a
pulmonologist (a lung specialist) is consulted during the diagnosis and
treatment of idiopathic pulmonary fibrosis. Some doctors suggest a lung transplant surgeon be consulted
when the patient is diagnosed with idiopathic pulmonary fibrosis unless there are contraindications
(Lung transplant may be more harmful to some people.) for
Because of the high incidence of gastroesophageal reflux (GERD)
in patients with idiopathic pulmonary fibrosis, a gastroenterologist may be consulted.
How is the diagnosis of idiopathic pulmonary fibrosis (IPF) made?
The diagnosis of idiopathic pulmonary fibrosis is somewhat difficult. It is a diagnosis of exclusion of
other known causes of interstitial lung disease including environmental and drug
exposures. There are no laboratory studies that are definitive, although up to
30% of people with idiopathic pulmonary fibrosis may have positive tests for antinuclear antibodies and/or
High resolution computed tomography (HRCT) is somewhat more specific and
sensitive for idiopathic pulmonary fibrosis diagnoses although not definitive, the same is true for
pulmonary function tests. However, repeated pulmonary function tests that show
declines over six months, when taken into account with other non-specific
findings, add to the possible diagnosis. However, a pattern of usual
interstitial pneumonia (UIP) on HRCT requires four features for diagnosis of
- Subplural, basal predominance of ground
- Reticular abnormalities
- Honeycombing with or without traction
- Absence of features listed as
inconsistent with UIP
Bronchoscopy and surgical lung biopsy are also used to help distinguish
between UIP and idiopathic pulmonary fibrosis. Consequently, the newest diagnostic criteria of
idiopathic pulmonary fibrosis
requires the following:
- Exclusion of other known causes of
interstitial lung disease (environmental exposures, connective tissue disease,
and/or drug toxicities)
- The presence of a UIP pattern,
described above, on HRCT in patients not subjected to surgical biopsy
- Specific combinations of HRCT and
surgical lung biopsy patterns in patients subjected to surgical lung biopsy
What are the treatment guidelines for treating idiopathic pulmonary fibrosis
(IPF) symptoms and signs?
The treatment for idiopathic pulmonary fibrosis depends upon the stage of the
disease, and usually begins with medical treatment, supportive measures, and
possibly surgical transplantation of the lungs. Often, during the mild to moderate disease medical care is initiated.
However, idiopathic pulmonary fibrosis sometimes progresses rapidly, so if
idiopathic pulmonary fibrosis is diagnosed, a transplant
surgeon should be consulted early as the person with IPF may eventually require lung
Guidelines on the treatment of idiopathic pulmonary fibrosis have been published by
several institutions including the American, European, Japanese, Latin American
thoracic societies and/or associations. The following is an abbreviated summary
of these guidelines:
- They conditionally recommend the use of
pirfenidone and nintedanib, both approved by the FDA in 2014.
- They do not conditionally recommend the
(Warfarin, Jantoven), combination prednisone with N-acetylcysteine, and
several other drugs.
- They conditionally recommend anti-acid
medications even in idiopathic pulmonary fibrosis patients without symptoms of reflux.
- Encourage the patient to quit any
- Encourage vaccination against influenza
and pneumococcal infections.
- They suggest patients with hypoxemia
(low oxygen in the blood, for example, pulse oximetry measured less than 88%)
use oxygen therapies to maintain a saturation at least 90% during all
activities, including sleeping.
Patients should discuss with their doctors on how this progressive disease
is best treated on an individual basis.
How effective is lung transplant for idiopathic pulmonary fibrosis (IPF)?
Lung transplantation is considered when the patient develops severe
stage (also termed end stage) idiopathic pulmonary fibrosis. Lung transplantation, does
show a survival benefit over medical therapy. Idiopathic pulmonary fibrosis has now replaced chronic
obstructive pulmonary disease (COPD)
as the most common reason for lung
transplantation in the US.
Any patient with idiopathic pulmonary fibrosis should be referred to a transplant surgeon to determine
early on if the patient may meet criteria for a lung transplant. Some people may
qualify for a single or a double lung transplant. Reported five-year survival
rates for lung transplantation are about 50% to 56%.
Criteria for a transplant vary somewhat so it is important to have the
patient and the medical team (primary care physician, pulmonologist, and
transplant surgeon) all agree on the potential criteria for the individual
Can idiopathic pulmonary fibrosis (IPF) be prevented?
Because idiopathic pulmonary fibrosis is "idiopathic," prevention is difficult. However, many
health-care professionals and researchers theorize that the risk of idiopathic pulmonary fibrosis may be reduced in
some people if they
quit smoking, and reduce or eliminate
exposure to toxins or fumes that have the potential to damage the lungs.
What is the life expectancy and prognosis for a person with idiopathic
pulmonary fibrosis (IPF)?
Idiopathic pulmonary fibrosis has a poor prognosis. The mean survival time or "life expectancy" is
about 2 to 5 years from the time of diagnosis. Most commonly, the person
experiences an acute exacerbation of idiopathic pulmonary fibrosis and then dies because of the disease.
Individuals with IPF who undergo lung transplant have a mortality rate of about 50% to 56% after five years so the transplant may increase the life expectancy in
some people with the disease.
Where can individuals with idiopathic pulmonary fibrosis (IPF) and their families and friends find support?
There are support groups for idiopathic pulmonary fibrosis patients and their families, for example,
the Pulmonary Fibrosis Foundation (www.pulmonaryfibrosis.org)
and the American Lung Association (www.lung.org).
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Medically Reviewed on 8/29/2016
Godfrey, A., MD. "Idiopathic Pulmonary Fibrosis." Medscape. Updated: Aug 11, 2016.
Kolb, M. et al. "Staging of idiopathic pulmonary fibrosis: past, present and future." European Respiratory Review. 23:220–224, 2014.