With a progressive character and permanent consequences, idiopathic pulmonary fibrosis affects the function of the lungs in a profound way (causing, for example, dyspnea) and is responsible for serious complications (such as pulmonary hypertension, cor pulmonale or lung cancer) .
Unfortunately, idiopathic pulmonary fibrosis is a condition for which only symptomatic treatments exist; this means, therefore, that the sick are destined to live with it, without any possibility of recovery.
Brief review of the term Fibrosis
In medicine, fibrosis is the term that identifies the abnormal formation of large amounts of connective-fibrous tissue (which is a functionless tissue, equivalent to scar tissue) in an organ of the human body, without any reference to the behavior of the parenchyma ( which is the functional tissue of an organ).
The processes of fibrosis alter the architecture of the target organ / tissue and this, combined with the absence of activity on the part of the connective-fibrous tissue, also affects its function.
Idiopathic pulmonary fibrosis is a progressive condition with irreversible consequences; in other words, it worsens over time and produces permanent changes in the lungs (where scar tissue is formed, the latter is stable and cannot be replaced with functional tissue).
What are the alveoli?
The alveoli, or pulmonary alveoli, are the small sacs located at the end of the intrapulmonary bronchial tree (secondary bronchi, tertiary bronchi, bronchioles, terminal bronchioles and respiratory bronchioles), in which the air introduced with inspiration and in which the human organism withdraws the oxygen contained in the aforementioned air. All around the alveoli, in fact, take place the blood capillaries that allow the blood, which flows internally, to release the carbon dioxide in exchange for the oxygen in the air.
The consequences
In people who develop idiopathic pulmonary fibrosis, the lungs become covered with scars, which have the effect of making them "harder" and less elastic and of "crushing" the alveoli as in a vise, preventing them from taking in the air. for the withdrawal of oxygen.
Classification of Idiopathic Pulmonary Fibrosis
From the didactic point of view, idiopathic pulmonary fibrosis belongs to the category of idiopathic interstitial pneumonia (non-infectious pneumonia, of unknown causes, affecting the lung interstitium), which, in turn, are part of the large pathological group of interstitial diseases of the lung (or diseases of the pulmonary interstitium).
For readers who are not aware of it, the "pulmonary interstitium (or simply interstitium) is the connective tissue interposed between the alveoli and appointed to give mechanical support to the latter."
Epidemiology
Idiopathic pulmonary fibrosis is a rare lung disease, which however has the distinction of being the most common variant of idiopathic interstitial pneumonia.
According to some statistical research, its annual incidence would fluctuate between 6.8 and 16.3 people per 100,000, in the USA, and between 4.6 and 7.4 per 100,000, in Europe.
More common among men than among women, idiopathic pulmonary fibrosis tends to preferably affect people over the age of 50.
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- A history of frequent exposure to metal, wood, coal, silica, stone and / or hay dusts;
- The so-called gastroesophageal reflux disease;
- A past history of viral infections caused by, for example, the Epstein-Barr virus or the hepatitis C virus.
It is important to remember, then, that, numbers in hand, idiopathic pulmonary fibrosis is more common among men (as if they were more predisposed to the lung disease in question) in old age.
of idiopathic pulmonary fibrosis are:- Dyspnea, i.e. difficulty in breathing;
- Dry cough;
- Chest pain
- Recurring feeling of fatigue and weakness;
- Weight loss for no reason
- Drumstick fingers (or hippocratic fingers and digital hippocratism);
The way of manifesting themselves and the severity of these manifestations vary from patient to patient: in some patients, the symptoms are important from the very beginning of the condition; in other patients, however, it becomes severe within a few months after an initial mild phase.
Did you know that ...
The severity of symptoms of idiopathic pulmonary fibrosis depends on how extensive the scar tissue is in the lung; in fact, as a rule, the more scar tissue there is, the worse the symptomatological picture.
Complications
Over time, people with idiopathic pulmonary fibrosis tend to develop complications, which further worsen their health, to the point of causing their death quite frequently and quickly enough.
Specifically, the possible complications of idiopathic pulmonary fibrosis include:
- Pulmonary hypertension. It is the abnormal and persistent rise in blood pressure inside the pulmonary arteries (ie the arterial vessels that carry oxygen-poor blood to the lungs) and inside the right cavities of the heart (hence the atrium and ventricle right).
Pulmonary hypertension is a very serious medical condition that tends to get worse over time and can lead to death.
In the presence of idiopathic pulmonary fibrosis, this condition depends on the compression that the scar tissue exerts on the alveoli and alveolar capillaries. - The pulmonary heart. It is a very serious heart disease, induced by "pulmonary hypertension and characterized by a" morphological alteration of the right ventricle of the heart (enlargement, very often combined with a thickening of the walls).
The appearance of cor pulmonale usually results in worsening of dyspnea. - Respiratory insufficiency. It is a severe morbid condition, in the presence of which the affected individual manifests severe shortness of breath and poor oxygenation of the blood (hypoxemia).
Respiratory failure generally constitutes the final stage of idiopathic pulmonary fibrosis. - Lung cancer. It is lung disease resulting from the uncontrolled growth of one of the constituent cells of the lungs.
In the presence of idiopathic pulmonary fibrosis, lung cancer represents a "possibility that further affects the patient's state of health and risks his life.
Report of Symptoms, Physical Examination and Anamnesis
The report of the symptoms, the physical examination and the anamnesis represent the preliminary diagnostic investigations, which help the doctor to know the symptoms in detail and to hypothesize all the possible causes.
Pulmonary Function Assessment Test
Performed routinely whenever a patient exhibits respiratory symptoms (e.g., dyspnoea, chest pain, etc.), pulmonary function tests are used to determine how the lungs are functioning and whether these symptoms are associated with impaired lung function.
In the diagnostic path that leads to the identification of idiopathic pulmonary fibrosis, the tests for the evaluation of pulmonary function are:
- Spirometry. Quick, practical and painless, it measures the inspiratory and expiratory capacity of the lungs; in addition, it provides information on the patency (or opening) of the pulmonary airways.
- Oximetry. It is the measurement of oxygen saturation in the blood. As simple and immediate as spirometry, for its execution you need an instrument called an oximeter, which is applied to a finger or to one of the two earlobes.
- The stress test. It consists in recording how the heart rhythm, blood pressure and breathing of the patient vary, while the patient is practicing a more or less intense physical activity.
Diagnostic for Images
Chest x-ray (or chest x-ray), chest CT scan, and chest MRI are three radiological tests that provide detailed images of the structures inside the chest (i.e. heart, lungs, great vessels, etc.).
In a hypothetical situation of idiopathic pulmonary fibrosis, this allows the diagnostician to assess the health of the lungs, identifying abnormalities such as the presence of abnormal scar tissue.
Lung Biopsy
Lung biopsy consists of taking (obviously from the patient) and subsequently analyzing in the laboratory a small sample of lung tissue.
The sampling can take place in at least 3 different ways: by bronchoscopy, by bronchoalveolar lavage and, finally, by surgery.
DRUGS
The only drugs valid for managing the symptoms of idiopathic pulmonary fibrosis and slowing its progression are corticosteroids (anti-inflammatory drugs) and immunosuppressants (drugs whose primary effect is to reduce the efficiency of the immune defenses).
Among the corticosteroids, prednisone deserves a mention; among the immunosuppressants, on the other hand, there are: methotrexate, cyclophosphamide, azathioprine, penicillamine and cyclosporine.
OXYGEN THERAPY
Oxygen therapy is the administration of extra oxygen, practical through special dispensers (some also portable). In general, its implementation takes place whenever there is a need to increase the amount of oxygen circulating in the blood.
In case of idiopathic pulmonary fibrosis, oxygen therapy produces the following benefits:
- Facilitates breathing;
- Reduces the risk of hypoxemia (low oxygen levels in the blood);
- Reduces blood pressure in the right cavities of the heart, preventing pulmonary hypertension and cor pulmonale;
- Improve night sleep.
Important: unfortunately oxygen therapy does not slow down the formation of scar tissue in the lungs.
RESPIRATORY REHABILITATION
Respiratory rehabilitation consists in having the patient practice a series of motor exercises (exercise bike, climbing stairs, walking, etc.), in order to improve tolerance to efforts and reduce the severity of dyspnea.
Important: like oxygen therapy, respiratory rehabilitation does not slow down the formation of scar tissue in the lungs.
LUNG TRANSPLANT
Lung transplantation is the surgery to replace a diseased lung with a healthy lung from a compatible donor.
Given the considerable invasiveness and the more than fair probability of failure of the operation (organ rejection), lung transplantation is an operation reserved for the most critical cases and when all the other solutions mentioned above have not provided any benefit.
LIFESTYLE
According to the reliable opinion of doctors, patients with idiopathic pulmonary fibrosis benefit:
- Do not smoke / stop smoking;
- Avoid secondhand smoke;
- Adopt a specific diet, which avoids the drop in body weight typical of those suffering from the lung disease in question;
- Get vaccinated against influenza (influenza vaccine) and pneumonia (pneumococcal vaccine).
