Idiopathic Pulmonary Fibrosis (IPF): Facts and Information
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Published: 2010-04-01 - Updated: 2023-01-29
Author: Thomas C. Weiss | Contact: Disabled World (Disabled-World.com)
Peer-Reviewed Publication: N/A
Library of Related Papers: Disability Information Publications
Synopsis: Information regarding Pulmonary Fibrosis including causes, symptoms, diagnosing, and IPF treatment options. Pulmonary Fibrosis is a condition in which the tissue deep in a person's lungs becomes thickened and stiff or scarred over some time. Approximately two-hundred thousand people in America alone have IPF; around fifty-thousand people are diagnosed with it yearly.
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 adults between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women. Approximately two-hundred thousand people in America alone have IPF; around fifty-thousand people are diagnosed with it yearly.
The U.S. Social Security Administration (SSA) has included Idiopathic Pulmonary Fibrosis as a Compassionate Allowance to expedite a disability claim.
- Pulmonary fibrosis
- Fibrosing alveolitis
- Usual interstitial pneumonitis
- Cryptogenic fibrosing alveolitis
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. The current thought is that an abnormal fibrotic and inflammatory response to microscopic injury results in scarring. There may also be genetic and epidemiological factors that 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.
Certain occupational exposures, or environmental ones, referred to as 'epidemiological factors,' appear to prevail in the medical histories of people diagnosed with IPF. Because of this, doctors may cite these exposures as factors contributing to a diagnosis of IPF. However, if there is a direct and clear relationship between these factors and the disease, the person's diagnosis would no longer be IPF. Epidemiological factors can include:
- Cigarette smoking
- Acid reflux disease
- Viral or bacterial lung infections
- Certain medicines such as antibiotics, anti-arrhythmias, anticonvulsants, chemotherapeutic agents and therapeutic radiation
- Prolonged exposure to dust such as asbestos, silica, beryllium and hard metal dust, organic dust such as bacteria and animal proteins
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, including rheumatoid arthritis, lupus, scleroderma, and sarcoidosis.
Medical science has an increasing amount of evidence suggesting that a family of genes may predispose certain people to IPF. Around ten-to-fifteen percent of people with IPF are considered 'familial,' something that is suggestive of a genetic predisposition. Recent studies related to IPF have found a mutation in the SP-C protein in families with a history of more than two people who have experienced IPF. Another study suggests that 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 cause pulmonary fibrosis in certain people as they age. Additional causes of IPF may include:
- Heart failure
- Lung cancer
- Pulmonary embolism
- Pulmonary hypertension
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. Eating and speaking on the phone become difficult or even impossible. Additional symptoms of IPF can include:
- Chest pain
- Loss of appetite
- Rapid weight loss
- Fatigue and weakness
- Discomfort in the chest
- Chronic dry, hacking cough
- Shortness of breath during activity
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, pulse oximeter testing, arterial blood gas testing, bronchoscopy, Bronchoalveolar lavage, Surgical Lung Biopsy, or Exercise testing about 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 any occupational, environmental, familial, or additional medical conditions might have contributed to 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 when the person was born or due to heart disease.
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 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 appropriately. The tests are commonly performed in either a clinical or hospital laboratory, consisting of breathing into a spirometer and sometimes in a box resembling 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 and 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 patterns 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 direct measurement of arterial pH, carbon dioxide, and oxygen through a direct arterial puncture. Their lungs have recently oxygenated the blood in a person's artery; 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 extracted.
A pulse oximeter indicates the amount of oxygen in the person's blood and is a device 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 between ninety-five and one-hundred percent on room air. Pulse oximetry does not measure carbon dioxide levels, so a blood gas measurement might be required in some persons.
Exercise testing usually involves a six-minute walk test. Exercise testing measures how well the person's lungs respond to exertion. Hospitals vary in their exercise testing methods, although it commonly involves using a treadmill or stationary bike. The person's oxygen saturation levels, blood pressure, and EKG are monitored as they exercise.
A bronchoscopy allows a doctor 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 often 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 to determine whether a person has IPF. Several diseases closely resemble IPF, with large differences in the treatment options and prognosis between them; the person being treated must receive the correct diagnosis. In combination with an HRCT, a surgical lung biopsy can also help a doctor determine the disease's progression. Obtaining a biopsy sample involves a minimally invasive procedure using video-assisted thoracoscopic surgery, which people usually tolerate well, although the procedure is not recommended for everyone.
Treatment of IPF
Medical science has not discovered a cure for IPF. Several people live between three and five years before receiving a diagnosis, with the most common cause of death related to IPF is 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. In 2009, at the Cleveland Clinic, the University of Pittsburgh Medical Center found that greater than fifty percent of lung transplants were 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 seventy to receive such transplants. Surgical techniques and outcomes have improved, and more people over seventy receive these transplants, with medical centers updating their age requirements to include people over seventy.
Facts and Statistics
- Although rare, IPF is the most common form of IIP.
- IPF is more common in men than women and is usually diagnosed in people over 50.
- A recent study from the USA estimated the incidence of IPF to be between 6.8 and 16.3 per 100,000 persons.
- The prevalence of IPF has been estimated between 14.0 and 42.7 per 100,000 persons based on a USA analysis of healthcare claims data, with variation depending on the case definitions used in this analysis.
- In the 27 European Union countries, a range of sources estimate an incidence of 4.6 to 7.4 people per 100,000, suggesting that approximately 30,000 to 35,000 new patients will be diagnosed with IPF each year.
Thomas C. Weiss is a researcher and editor for Disabled World. Thomas attended college and university courses earning a Masters, Bachelors and two Associate degrees, as well as pursing Disability Studies. As a Nursing Assistant Thomas has assisted people from a variety of racial, religious, gender, class, and age groups by providing care for people with all forms of disabilities from Multiple Sclerosis to Parkinson's; para and quadriplegia to Spina Bifida.
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