IDEOPATHIC PULMONARY FIBROSIS
http://www.ipfworld.org/#
Idiopathic Pulmonary Fibrosis (IPF)
IPF is one specific kind of PF. The word "idiopathic" means that we don't know the cause of the disease. But we don't use the term IPF to refer to the all-to-common situation of not being able to find a cause for your PF. Instead, we reserve the term IPF for one specific type of PF of unknown cause.
The fundamental problem in IPF is scarring that occurs in a predictable pattern in the lung. In IPF, scarring begins in the walls of the air sacs just beneath the outer lining of the lung (this outer lining is called the "pleura", so the location of the scarring is called "sub-pleural" by doctors) and predominates in the parts of the lung that are closest to the ground when you are standing up (the so-called "basilar" part of the lungs). So, in IPF, the scarring begins in the walls of subpleural basilar alveoli. As the disease progresses, the scar tissue spreads to involve more and more of the lung. Eventually, the lung is mostly replaced by scar tissue.
Four consequences of PF: crackles heard in the lungs; stiff lungs detected by pulmonary function testing; low oxygen levels in the blood particularly during exertion; and abnormalities seen on a CAT scan which are often quite characteristic (scar tissue in the subpleural basilar parts of the lungs) - this is called a "usual interstitial pneumonia" or UIP pattern on the CAT scan.
More cases generally occur in men than in women and it is usually diagnosed in adults who are aged between 40 and 80 years old.
Early symptoms: Chronic cough: usually a dry cough that doesn’t produce mucus.
Shortness of breath that occurs while performing physical activities.
Late symptoms: Shortness of breath and/or episodes of coughing, while at rest, that affect a person’s ability to eat, talk on the telephone, or walk up and down steps.
Enlargement of the fingertips, and thickening of the nail beds with a spoon-shaped appearance called “clubbing” occurs in about half patients. Sometimes fluid retention in the legs and arms (peripheral oedema).
Progression cannot be readily predicted even when the diagnosis has been made. A unique aspect of IPF is that the medical condition can deteriorate in sudden, unexpected jumps − doctors speak of “acute exacerbations”. These IPF exacerbations are marked by an intense shortness of breath, violent coughing episodes and fatigue.
IPF is a specific type of pulmonary fibrosis in which the small air sacs of the lung, known as “alveoli”, gradually become replaced by fibrotic (scar) tissue. The scarring begins in the tissue between the air sacs. Normally, this tissue is composed of a thin layer of cells, but scarring with IPF causes the tissue to thicken and become stiff, preventing oxygen from getting into the bloodstream. Over time, this scarring of the lungs worsens, the lungs become stiffer and breathing becomes more difficult. A person will find that they become increasingly short of breath. The actual cause of IPF is currently unknown. This distinguishes IPF from other forms of lung fibrosis for which a definite cause is known and better understood, such as exposure to a harmful substance like asbestos.
How is the diagnosis made?
There are many types of lung disease that can produce symptoms similar to those of IPF. For this reason several tests are used to make a diagnosis
Physical examination
The majority of patients who are eventually diagnosed with IPF first visit their doctor because they have a dry cough or shortness of breath. When listening to the lungs (auscultation), the doctor can often hear a crackling sound in both lungs at the end of inhalation. A lack of oxygen in the bloodstream can lead to a violet or bluish coloration of the skin and fingernails.
Imaging procedures
Chest X-rays
It is harder for X-rays to pass through fibrotic lung tissue than through healthy lungs. The changes in lung tissue that are seen in IPF patients become visible on X-ray images as white areas in the places where the X-ray beam is reflected away, instead of easily passing through the lung itself. These thickened white areas cannot be easily seen in the X-ray pictures of all patients and, once observed, a radiologist may want to study them in greater detail using more sophisticated techniques, such as a high resolution computer tomography (HRCT) scan.
High resolution computer tomography (HRC
HRCT scans of the chest are very precise and they make it possible to recognize IPF disease, as well as other chest abnormalities, at an earlier stage. Computer tomography pictures can be taken in many, very thin, slices and this allows for highly detailed and accurate mapping of the chest area. This method ensures that any changes or abnormalities will be recognized with greater accuracy. The HRCT images of patients with idiopathic lung fibrosis typically appear with “open spaces” inside the lung, with areas along the edges of the chest wall that look like honeycombs.
Lung function tests
Lung function tests are performed to measure a person’s breathing capacity, to determine the “stiffness” of the lungs, and to calculate the amount of oxygen that transfers from the lungs into the bloodstream. These can be done easily, and are very accurate ways of diagnosing current lung function, but they can also be used to follow a patient’s condition over time. These are non-invasive tests.
Bronchoscopy (lung endoscopy)
A bronchoscope is a flexible tube with a camera attached to the end that a pneumologist uses to visually examine the airways. This examination is usually conducted with sedation and is not painful. The bronchoscope is passed through the patient’s nose or mouth and advanced into the airway to look at the anatomy and, occasionally, to collect samples of lung tissue, fluid or foreign material, such as infections. With the bronchoscope it is possible to obtain a specimen of secretions from the lungs so that these can be examined microscopically and microbiologically.
Biopsy
If the above tests do not lead to a clear diagnosis for the patient, a surgical procedure can be performed to take further samples of lung tissue for subsequent examination under a microscope. In virtually all cases, this will allow a further, detailed assessment of the disease, and can result in a reliable diagnosis. Today, this procedure can almost always be performed by means of gentle, keyhole technology using a video-assisted thoracoscopy (VATS). This procedure is performed under general anaesthesia and is the least invasive way of taking a biopsy of the lungs.
Vaccinations
IPF patients often suffer from respiratory tract infections. Therefore, older patients should be sure to have regular vaccinations. A vaccination against the pneumonia (pneumococcus) infection is worth considering, as is a flu shot.
http://www.ipfworld.org/#
Idiopathic Pulmonary Fibrosis (IPF)
IPF is one specific kind of PF. The word "idiopathic" means that we don't know the cause of the disease. But we don't use the term IPF to refer to the all-to-common situation of not being able to find a cause for your PF. Instead, we reserve the term IPF for one specific type of PF of unknown cause.
The fundamental problem in IPF is scarring that occurs in a predictable pattern in the lung. In IPF, scarring begins in the walls of the air sacs just beneath the outer lining of the lung (this outer lining is called the "pleura", so the location of the scarring is called "sub-pleural" by doctors) and predominates in the parts of the lung that are closest to the ground when you are standing up (the so-called "basilar" part of the lungs). So, in IPF, the scarring begins in the walls of subpleural basilar alveoli. As the disease progresses, the scar tissue spreads to involve more and more of the lung. Eventually, the lung is mostly replaced by scar tissue.
Four consequences of PF: crackles heard in the lungs; stiff lungs detected by pulmonary function testing; low oxygen levels in the blood particularly during exertion; and abnormalities seen on a CAT scan which are often quite characteristic (scar tissue in the subpleural basilar parts of the lungs) - this is called a "usual interstitial pneumonia" or UIP pattern on the CAT scan.
More cases generally occur in men than in women and it is usually diagnosed in adults who are aged between 40 and 80 years old.
Early symptoms: Chronic cough: usually a dry cough that doesn’t produce mucus.
Shortness of breath that occurs while performing physical activities.
Late symptoms: Shortness of breath and/or episodes of coughing, while at rest, that affect a person’s ability to eat, talk on the telephone, or walk up and down steps.
Enlargement of the fingertips, and thickening of the nail beds with a spoon-shaped appearance called “clubbing” occurs in about half patients. Sometimes fluid retention in the legs and arms (peripheral oedema).
Progression cannot be readily predicted even when the diagnosis has been made. A unique aspect of IPF is that the medical condition can deteriorate in sudden, unexpected jumps − doctors speak of “acute exacerbations”. These IPF exacerbations are marked by an intense shortness of breath, violent coughing episodes and fatigue.
IPF is a specific type of pulmonary fibrosis in which the small air sacs of the lung, known as “alveoli”, gradually become replaced by fibrotic (scar) tissue. The scarring begins in the tissue between the air sacs. Normally, this tissue is composed of a thin layer of cells, but scarring with IPF causes the tissue to thicken and become stiff, preventing oxygen from getting into the bloodstream. Over time, this scarring of the lungs worsens, the lungs become stiffer and breathing becomes more difficult. A person will find that they become increasingly short of breath. The actual cause of IPF is currently unknown. This distinguishes IPF from other forms of lung fibrosis for which a definite cause is known and better understood, such as exposure to a harmful substance like asbestos.
How is the diagnosis made?
There are many types of lung disease that can produce symptoms similar to those of IPF. For this reason several tests are used to make a diagnosis
Physical examination
The majority of patients who are eventually diagnosed with IPF first visit their doctor because they have a dry cough or shortness of breath. When listening to the lungs (auscultation), the doctor can often hear a crackling sound in both lungs at the end of inhalation. A lack of oxygen in the bloodstream can lead to a violet or bluish coloration of the skin and fingernails.
Imaging procedures
Chest X-rays
It is harder for X-rays to pass through fibrotic lung tissue than through healthy lungs. The changes in lung tissue that are seen in IPF patients become visible on X-ray images as white areas in the places where the X-ray beam is reflected away, instead of easily passing through the lung itself. These thickened white areas cannot be easily seen in the X-ray pictures of all patients and, once observed, a radiologist may want to study them in greater detail using more sophisticated techniques, such as a high resolution computer tomography (HRCT) scan.
High resolution computer tomography (HRC
HRCT scans of the chest are very precise and they make it possible to recognize IPF disease, as well as other chest abnormalities, at an earlier stage. Computer tomography pictures can be taken in many, very thin, slices and this allows for highly detailed and accurate mapping of the chest area. This method ensures that any changes or abnormalities will be recognized with greater accuracy. The HRCT images of patients with idiopathic lung fibrosis typically appear with “open spaces” inside the lung, with areas along the edges of the chest wall that look like honeycombs.
Lung function tests
Lung function tests are performed to measure a person’s breathing capacity, to determine the “stiffness” of the lungs, and to calculate the amount of oxygen that transfers from the lungs into the bloodstream. These can be done easily, and are very accurate ways of diagnosing current lung function, but they can also be used to follow a patient’s condition over time. These are non-invasive tests.
Bronchoscopy (lung endoscopy)
A bronchoscope is a flexible tube with a camera attached to the end that a pneumologist uses to visually examine the airways. This examination is usually conducted with sedation and is not painful. The bronchoscope is passed through the patient’s nose or mouth and advanced into the airway to look at the anatomy and, occasionally, to collect samples of lung tissue, fluid or foreign material, such as infections. With the bronchoscope it is possible to obtain a specimen of secretions from the lungs so that these can be examined microscopically and microbiologically.
Biopsy
If the above tests do not lead to a clear diagnosis for the patient, a surgical procedure can be performed to take further samples of lung tissue for subsequent examination under a microscope. In virtually all cases, this will allow a further, detailed assessment of the disease, and can result in a reliable diagnosis. Today, this procedure can almost always be performed by means of gentle, keyhole technology using a video-assisted thoracoscopy (VATS). This procedure is performed under general anaesthesia and is the least invasive way of taking a biopsy of the lungs.
Vaccinations
IPF patients often suffer from respiratory tract infections. Therefore, older patients should be sure to have regular vaccinations. A vaccination against the pneumonia (pneumococcus) infection is worth considering, as is a flu shot.