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Idiopathic Pulmonary Fibrosis (IPF): Facts and Information

  • Published: 2010-04-01 (Revised/Updated 2017-06-26) : Thomas C. Weiss (Disabled World).
  • Synopsis: Pulmonary Fibrosis is when tissue deep in the lungs becomes thickened and stiff or scarred over a period of time.
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. Fibrosis is usually associated with a poor prognosis. The term idiopathic is used because the cause of pulmonary fibrosis is still unknown. IPF usually occurs in adult individuals of between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women.

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Quote: "A number of people live between three and five years beyond the time of receiving a diagnosis, with the most common cause of death related to IPF being respiratory failure."

Approximately two-hundred thousand people in America alone have IPF; around fifty-thousand people are diagnosed with it every year.

Defining IPF

Pulmonary Fibrosis is a condition in which the tissue deep in a person's lungs become thickened and stiff or scarred over a period of time. Scar tissue that develops is referred to as, 'fibrosis.' As the person's lung tissue thickens, they it loses the ability to move oxygen into their bloodstream. The result is a lack of oxygen circulating to the person's brain and additional organs in their body. For some people, a doctor is able to discover the cause of fibrosis. In the majority of persons with pulmonary fibrosis however, doctors are unable to specify a cause. When this is the case, the person is diagnosed with, 'Idiopathic Pulmonary Fibrosis (IPF).'

Approximately two-hundred thousand people in America alone have IPF; around fifty-thousand people are diagnosed with it every year. IPF affects mostly people between the ages of fifty and seventy-five years of age. IPF is a serious condition that varies among individuals; in some people their lung tissue thickens and becomes stiff quickly, while in others the process is slow. For still others, their condition remains the same for years. IPF is also known by the names:

Causes of IPF

Medical science has not found the causes of IPF, although it is believed to be the result of an inflammatory response to an unknown substance. Current thought is that there is an abnormal fibrotic and inflammatory response to microscopic injury that ends up resulting in scarring. There may also be genetic and epidemiological factors which contribute to the development of IPF. Further definition of these factors could lead to a better understanding of the disease process, leading to treatment options.

There are certain occupational exposures, or environmental ones, referred to as, 'epidemiological factors,' that appear to prevail in the medical histories of people who have been diagnosed with IPF. Because of this, doctors may cite these exposures as factors that contribute to a diagnosis of IPF. If there is a direct and clear relationship between these factors and the disease; however, the person's diagnosis would no longer be IPF. Epidemiological factors can include:

IPF might be associated with upper respiratory infections, such as tuberculosis or pneumonia. The particular connection between IPF and these diseases is unknown. Interstitial lung diseases in general have been associated with connective tissue diseases, to include rheumatoid arthritis, lupus, scleroderma, and sarcoidosis.

Medical science has an increasing amount of evidence suggesting that there is a family of genes that may predispose certain people to IPF. Around ten-to-fifteen percent of people with IPF are considered to be, 'familial,' something that is suggestive of a genetic predisposition. Recent studies related to IPF have found a mutation in the SP-C protein that exists in families with a history of more than two people who have experienced IPF. There is another study which suggests that the presence of specific genes might predict which people with IPF will experience a more severe and rapid progression of the disease. Still another study demonstrated that shortened, 'telomeres,' which protect the ends of chromosomes from deterioration, could be the cause of pulmonary fibrosis in certain people as they age. Additional causes of IPF may include:

Symptoms of IPF

The symptoms of IPF are not always present at the onset of the disease. The symptoms of IPF may not appear until the disease has progressed. The main symptom of IPF is shortness of breath or, 'dyspnea,' something many people describe as,' breathlessness.' Many people ignore occasional breathing difficulties, putting it down to simple aging or being out of shape. As IPF progresses and damage to the person's lungs increases in severity, the dyspnea associated with IPF increases, potentially making even minor physical activities such as getting dressed or taking a shower difficult. Activities such as eating and speaking on the phone become difficult or even impossible. Additional symptoms of IPF can include:

Diagnosing IPF

Doctors have various ways to achieve a diagnosis of IPF depending upon the person's medical history and the symptoms the person is experiencing. A doctor can pursue the person's history, a physical examination, a chest X-ray, a high-resolution computerized tomography, pulmonary function testing, pulseoximeter testing, arterial blood gas testing, bronchoscopy, Bronchoalveolar lavage, Surgical Lung Biopsy, or Exercise testing in relation to IPF. A doctor will usually pursue one or more of these tests.

History and Physical Exam: The person's doctor will usually obtain a detailed medical history of the person involved in an attempt to learn if there are any occupational, environmental, familial, or additional medical conditions that might have contributed or predisposed them to the development of IPF. The doctor will listen to the person's lungs; they might hear crackling sounds through their stethoscope, indicative of openings made by small airways as the person breathes in. Approximately fifty-percent of persons with IPF have, 'clubbing,' of their fingertips, a widening of their fingertips caused by a lack of oxygen in their blood. The clubbing is not specific to IPF; it does happen with other lung disorders and may also be present from the time the person was born, or due to heart disease.

Chest X-Ray: A doctor may order a chest X-ray as a screening test. Five-to-fifteen percent of persons with significant scarring will still present with an average chest X-ray; IPF is something that cannot be diagnosed through a chest X-ray alone.

Pulmonary Function Tests: Breathing tests may be performed to measure the person's lungs ability to exchange oxygen and carbon dioxide in an appropriate manner. The tests are commonly performed in either a clinical or hospital laboratory, consisting of breathing into a spirometer and at times performed in a box that resembles a glass telephone booth. One of the components of a Pulmonary Function Test involves Spirometry, which measures the person's inspired and exhaled lung volumes as well as the rate at which they occur, another component measures the ability of oxygen to diffuse into the person's bloodstream.

High Resolution Computerized Tomography (HRCT): A doctor may order this form of testing, which provides a sharp and detailed image of the person's lungs, in an attempt to identify specific clinical patters in their lung tissue that are indicative of disease in IDF, such as a honeycombing pattern suggesting lung scarring and damage to the person's air sacs, or a hazy appearance to their lung tissue that is associated with inflammation.

Arterial Blood Gas (ABG): ABG involves a direct measurement of arterial pH, carbon dioxide, and oxygen through a direct arterial puncture. The blood in a person's artery has been recently oxygenated by their lungs; it therefore indicates how much oxygen is available to their body. Blood in a person's veins has a lower oxygen concentration and indicates the amount of oxygen that has been extracted.

Pulse Oximeter: A pulse oximeter indicates the amount of oxygen in the person's blood, and is a device that is placed on the person's finger or earlobe. A pulse oximeter transmits light at different wavelengths through the person's small blood vessels. Average ranges are considered to be between ninety-five and one-hundred percent on room air. Pulse oximetry does not; however, measure carbon dioxide levels, so a blood gas level measurement might be required in some persons.

Exercise testing: Exercise testing usually involves a six-minute walk test. Exercise testing is used in order to measure how well the person's lungs respond to exertion. Hospitals vary in the methods they use for exercise testing, although it commonly involves the use of a treadmill or stationary bike. The person's oxygen saturation levels, blood pressure, and EKG are monitored as they exercise.

Bronchoscopy: A bronchoscopy gives a doctor the ability to examine a person's main airways of their lungs via the use of a small and flexible tube called a, 'bronchoscope.' Bronchoscopy can help a doctor to evaluate problems with a person's lungs, or examine blockages to them; it also provides a way to collect fluid or tissue samples. The lung tissue samples that may be obtained through the procedure; however, are oftentimes small and inadequate for use in reaching a definitive diagnosis.

Surgical Lung Biopsy: A surgical lung biopsy is the most revealing diagnostic tool available to a doctor in determining whether or not a person has IPF. There are a number of diseases that closely resemble IPF, with large differences in the treatment options and prognosis between them; it is vital that the person being treated receive the correct diagnosis. In combination with an HRCT, a surgical lung biopsy can help a doctor to determine the progression of the disease as well. Obtaining a biopsy sample involves a minimally invasive procedure using video-assisted thoracoscopic surgery; something that people usually tolerate well, although the procedure is not recommended for everyone.

Treatment of IPF

Medical science has not discovered a cure for IPF. A number of people live between three and five years beyond the time of receiving a diagnosis, with the most common cause of death related to IPF being respiratory failure. There are no forms of medications that have demonstrated the ability to improve the outcome of people with the condition, although for some who have IPF, medications such as cytotoxic drugs or corticosteroids might help to reduce inflammation.

Oxygen use can help people with IPF to increase the amount of oxygen in their blood. While lung rehabilitation will not cure IPF, it can assist in maintaining the person's exercise ability. Some people with IPF might need a lung transplant.

Idiopathic Pulmonary Fibrosis (IPF) has become the leading indication for lung transplantation in most large centers. The year 2009 at the Cleveland Clinic, University of Pittsburgh Medical Center, found greater than fifty-percent of the lung transplants performed due to IPF. Lung transplantation may improve the quality of the affected person's life and longevity, if they do not have other significant health issues. In the past it was uncommon for people over the age of seventy to receive such transplants. Surgical techniques and outcomes have improved, and more people over the age of seventy are receiving these transplants because of it, with medical centers updating their age requirements to include people over the age of seventy.

Awareness: Pulmonary Fibrosis Awareness

Red and blue awareness ribbonThe Pulmonary Fibrosis (PF) Awareness ribbon is blue and red in color.

The month of September is Global Pulmonary Fibrosis (PF) Awareness Month.

Statistics: Idiopathic Pulmonary Fibrosis

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