Idiopathic Pulmonary Fibrosis by A.Griguolo

Generality

Idiopathic pulmonary fibrosis is a lung disease of unknown causes, characterized by the anomalous formation of scar tissue all around the alveoli.

With a progressive character and permanent consequences, idiopathic pulmonary fibrosis affects lung function profoundly (causing, for example, dyspnea) and is responsible for serious complications (such as pulmonary hypertension, pulmonary heart or lung cancer) .

Unfortunately, idiopathic pulmonary fibrosis is a condition for which there are only symptomatic treatments; 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 anomalous formation of large quantities of connective-fibrous tissue (which is a tissue without function, 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 fibrosis processes alter the architecture of the target organ / tissue and this, combined with the absence of activity by the connective-fibrous tissue, also affects its function.

What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a chronic type of lung disease (thus a long-lasting lung disease), marked by abnormal formation and for unknown reasons of scar tissue all around the alveoli .

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 forms, the latter is stable and not replaceable with functional tissue).

What are 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 by inhalation ends in which takes place the withdrawal, by the human body, of 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 carbon dioxide in exchange for oxygen from 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 if in a vice, preventing them from retaining the air for the withdrawal of oxygen.

Classification of Idiopathic Pulmonary Fibrosis

From an educational point of view, idiopathic pulmonary fibrosis belongs to the category of idiopathic interstitial pneumonia (non-infectious pneumonias, with unknown causes, which affect the pulmonary interstitium ), which, in turn, are part of the large pathological group of interstitial diseases of the lung (or pulmonary interstitial diseases ).

For readers who are not aware of it, the interstitium (or simply interstitium) is the connective tissue interposed between the alveoli and deputed to give mechanical support to the latter.

Epidemiology

Idiopathic pulmonary fibrosis is a rare lung disease, but it has the primacy of being the most common variant of idiopathic interstitial pneumonia.

According to some statistical research, its annual incidence would range between 6.8 and 16.3 people per 100, 000, in the US, and between 4.6 and 7.4 per 100, 000, in Europe.

More common among men than women, idiopathic pulmonary fibrosis tends to affect people over the age of 50 preferably.

Causes

Despite numerous research on the subject, the causes of idiopathic pulmonary fibrosis are unknown; the adjective "idiopathic" refers precisely to this lack of knowledge related to causal factors.

Risk Factors of Idiopathic Pulmonary Fibrosis

The various studies conducted so far on the causal origin of idiopathic pulmonary fibrosis have not been completely useless. Indeed, from these investigations it emerged that idiopathic pulmonary fibrosis is more frequent in the presence of:

A family history of pulmonary fibrosis or pulmonary interstitial disease. Research shows that more than 20% of people with idiopathic pulmonary fibrosis belong to a family, where there is or was another member with the same disease or, however, a form of idiopathic interstitial pneumonia;
The habit of cigarette smoking . According to statistics, more than 75% of people with idiopathic pulmonary fibrosis have a history of smoking;

A history of frequent exposure to metal, wood, coal, silica, stone and / or hay dust ;
The so-called gastroesophageal reflux disease ;
A past history of viral infections supported, for example, by Epstein-Barr virus or hepatitis C virus .

It is important to remember, then, that, numbers in hand, idiopathic pulmonary fibrosis is more common among men (as if these were more predisposed to the lung disease in question) at advanced age.

Symptoms and Complications

The typical symptoms and signs of idiopathic pulmonary fibrosis are:

Dyspnea, or difficulty breathing;
Dry cough;
Chest pain;

Recurrent feeling of fatigue and weakness;
Weight loss for no reason;
Drumstick fingers (or Hippocratic fingers and digital hippocratism);

The way of manifesting and the gravity of such manifestations vary from patient to patient: in some patients, the symptomatology is important from the beginning of the condition; in other patients, however, it becomes severe within a few months after a mild initial phase.

Did you know that ...

The severity of the symptoms of idiopathic pulmonary fibrosis depends on how extensive the scar tissue is at the lung level; in fact, in fact, the more scar tissue there is, the worse the symptomatic picture.

Complications

Over time, patients with idiopathic pulmonary fibrosis tend to develop complications, which further worsen their state of health, to the point that they cause death with some frequency and in fairly rapid times.

Specifically, the possible complications of idiopathic pulmonary fibrosis include:

Pulmonary hypertension . It is the abnormal and persistent raising of blood pressure within the pulmonary arteries (ie arterial vessels that carry oxygen-poor blood to the lungs) and inside the right cavities of the heart (ie right atrium and ventricle).
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 on the 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 thickening of the walls).
The appearance of the pulmonary heart usually leads to worsening dyspnea.
Respiratory failure . It is a severe morbid condition, in the presence of which the individual concerned shows significant shortage of breath and poor blood oxygenation (hypoxemia).
Respiratory failure generally constitutes the final stage of idiopathic pulmonary fibrosis.
Lung cancer . It is the lung disease resulting from the uncontrolled growth of one of the lung's constituent cells.
In the presence of idiopathic pulmonary fibrosis, lung cancer represents an eventuality that further undermines the health status of the patient and that risks his life.

Diagnosis

In general, to formulate the diagnosis of idiopathic pulmonary fibrosis and establish the extent of this lung disease, physicians need information from: the patient's symptoms, physical examination, medical history, tests for pulmonary function assessment, lung imaging (chest X-ray, chest magnetic resonance, and / or chest CT) and lung biopsy .

Tale of Symptoms, Objective Exam and History

The story of the symptoms, the physical examination and the medical history represent the preliminary diagnostic investigations, which help the doctor to know the symptoms in detail and to hypothesize all the possible causes.

Pulmonary Functionality Assessment Test

Performed routinely whenever a patient exhibits respiratory symptoms (eg, dyspnea, chest pain, etc.), tests to assess lung function are used to determine how the lungs work and whether the above symptoms are associated with a deficit in pulmonary functioning.

In the diagnostic pathway that leads to the identification of idiopathic pulmonary fibrosis, the tests for the evaluation of the expected lung function are:

Spirometry . Fast, practical and painless, it measures the inspiratory and expiratory capacity of the lungs; furthermore, it provides information regarding 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 ear lobes.
The stress test . It consists of recording how the heart's rhythm, blood pressure and patient's breathing vary, while the latter is practicing more or less intense physical activity.

Diagnostics for Images

Chest X-rays (or chest X-rays), chest CT scans, and chest MRI are three radiological examinations that provide detailed images of the internal structures of the chest (ie heart, lungs, large vessels, etc.).

In a hypothetical situation of idiopathic pulmonary fibrosis, this allows the diagnostic physician to assess the state of health of the lungs, identifying anomalies such as the presence of abnormal scar tissue.

Pulmonary Biopsy

The lung biopsy consists in taking (obviously from the patient) and then analyzing a small sample of lung tissue in the laboratory.

The sampling can be done in at least 3 different ways: by bronchoscopy, by bronchoalveolar lavage and, finally, by surgery .

Therapy

Idiopathic pulmonary fibrosis is treatable exclusively in terms of symptoms ; at the present time, in fact, there are neither cures capable of eliminating the anomalous scar tissue, nor even treatment able to stop the inexorable progression of the lung disease in question.

In essence, therefore, those suffering from idiopathic pulmonary fibrosis can only rely on symptomatic therapy and are destined to coexist with the aforementioned pathology.

Symptomatic Therapy: what does it consist of?

The symptomatic therapy of idiopathic pulmonary fibrosis presents, in fact, 3 objectives, which are:

Relieve symptoms;
Slow down the inexorable progression of the disease;
Postpone / avoid the most serious complications.

Coming therefore to the details, in the list of possible symptomatic treatments in the presence of idiopathic pulmonary fibrosis, there are: drugs, oxygen therapy, the so-called respiratory rehabilitation, lung transplantation and the adoption of a specific lifestyle .

DRUGS

Corticosteroids (anti-inflammatory drugs) and immunosuppressants (drugs whose primary effect is to reduce the efficiency of the immune defenses) are the only valid drugs to manage the symptoms of idiopathic pulmonary fibrosis and to slow its progression.

Among corticosteroids, prednisone deserves a mention; among the immunosuppressants, on the other hand, we note: 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 occurs whenever there is a need to increase the amount of oxygen circulating in the blood.

In the case of idiopathic pulmonary fibrosis, oxygen therapy produces the following benefits:

Facilitates breathing;
Reduces the risk of hypoxemia (low levels of oxygen in the blood);
Reduces blood pressure in the right cavities of the heart, preventing pulmonary hypertension and pulmonary heart;
Improves sleep at night.

Important : unfortunately oxygen therapy does not slow down the formation of scar tissue in the lungs in any way.

RESPIRATORY REHABILITATION

Respiratory rehabilitation consists in having the patient practice a series of motor exercises (exercise bikes, climbing stairs, walking etc.), in order to improve tolerance to efforts and reduce the severity of dyspnea.

Important : like oxygen therapy, even respiratory rehabilitation does not slow down the formation of scar tissue at the lung level.

LUNG TRANSPLANTATION

Lung transplantation is a surgical procedure 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 the doctors, patients with idiopathic pulmonary fibrosis benefit:

Do not smoke / stop smoking;
Avoid passive smoking;
Adopt a specific diet, which avoids the drop in body weight typical of those suffering from the lung disease in question;
Get vaccinated against the flu (flu vaccine) and against pneumonia (pneumococcal vaccine).

Prognosis

Being an incurable, progressive disease with permanent consequences, idiopathic pulmonary fibrosis has a poor prognosis.

Survival time

Several statistical studies have shown that, for most patients with idiopathic pulmonary fibrosis, the survival time after diagnosis is only 2-3 years .