What is it?
Within Interstitial Lung Diseases (PID), the most frequent is Idiopathic Pulmonary Fibrosis (IPF). This group is a heterogeneous set of pulmonary pathologies that affect the alveolar-interstitial structures and that present similar clinical-radiological characteristics.
In IPF, lung tissue undergoes an abnormal scarring process characterized by an excess of fibrotic tissue that gradually replaces healthy tissue. It is a progressive chronic disease that only affects the lungs of unknown cause, hence the name idiopathic, although it is believed that there are various risk factors that may be associated with its development.
Symptoms early in the course are nonspecific and only become apparent when fibrotic tissue has largely replaced healthy lung tissue and thus breathing is affected.
How is it produced?
Although the mechanism that causes fibrosis is not known for sure, it is thought that certain external stimuli cause damage to the epithelial-interstitial cells of the alveoli (small air sacs in which gas exchange takes place in inside the lungs). This lesion is repaired by an increase in chemicals and cells (microblasts and fibroblasts) that generate scar tissue without respiratory function. Fibrosis destroys and replaces healthy lung tissue, which prevents gas exchange and, therefore, the arrival of oxygen to the blood. Over time, and as the disease progresses, the lungs become more rigid and hard, making it difficult to breathe.
The factors associated with the development of this pathology are:
- Smoking: Smoking increases the chance of developing IPF
- Prolonged exposure to dust and occupational or environmental pollutants: Exposure to wood dust, metals, dust of animal and vegetable origin, among others, is related to an increased risk of suffering from IPF
- Viral or bacterial lung infections: Controversial
- Certain medications such as antibiotics, antirhythmics, anticonvulsants, antineoplastics, or radiation therapy.
- Gastroesophageal reflux: Microaspirations caused by abnormal gastric acid reflux are thought to be a risk factor for IPF
- Genetic predisposition: If there is a family history of IPF, the risk of suffering from it is increased.
Epidemiology
It is a pathology more frequent in men than in women and usually appears between the ages of 50 and 80.
In Spain it is estimated that the incidence is around 1.6 cases per 100.000 inhabitants.
symptomatology
The most frequent symptoms of the disease are:
- Dyspnea: The sensation of suffocation, in the initial phases, appears with intense effort and as the disease progresses it increases until it occurs at rest.
- Chronic and dry cough: It is not usually accompanied by secretions.
- Tiredness and asthenia.
- Chest discomfort: They are usually non-specific discomfort in the chest and back, frequently associated with intercostal muscle problems caused by coughing.
- Peripheral cyanosis: Bluish coloration of the skin and mucous membranes due to lack of oxygenation.
- Finger clubbing or clubbing.
- Anorexia.
- Unexplained weight loss.
- Edema in the lower limbs, hands and/or abdomen: Caused by fluid retention.
Evolution
The evolution of the disease does not follow a fixed pattern. There are patients in whom it is very slow and in others fast.
It is usually characterized by periods of stability followed by acute exacerbations that can be fatal in the most severe cases and worsen in milder cases, despite the disappearance of the exacerbation.
When the deterioration is very great, oxygen therapy and a lung transplant may be required.
Diagnosis
Establishing a diagnosis of IPF is complicated by the initial signs and symptoms of this pathology are very similar to those of other respiratory diseases. Once it is known that the patient suffers from Pulmonary Fibrosis, it is necessary to determine if it could be secondary to another pathology, thus ruling out that it is idiopathic. For this, a detailed clinical history will be carried out that includes:
- smoking history
- Family background
- Environmental exposure
- Work history
- Exposure to animals (birds, pigeons)
- Medication use
- Previous treatments with chemotherapy and/or radiotherapy
- previous infections
- Existence of some rheumatic diseases (rheumatoid arthritis, scleroderma, etc.).
This history will be complemented with a physical examination that includes lung auscultation, observation of the existence of cyanosis and clubbing, as well as laboratory tests and other diagnostic tests such as:
- Diagnostic imaging techniques:
- Chest X-ray: It allows to see the amount of fibrous lung tissue and its distribution.
- High Resolution Computed Axial Tomography (HAT): Essential test for the diagnosis of IPF, especially in the early stages.
- Lung function tests:
- Gasometry.
- pulse oximetry.
- Walk test 6 minutes.
- Respiratory function tests: They are used to study the evolution of the disease. These tests measure:
- Air flow: Spirometry
- lung volumes
- Carbon Diffusion Capacity
- Lung biopsy: Used for definitive diagnosis.
Treatment
Pharmacological
To this day there is no cure for IPF. With the treatment, what is intended is to improve the symptoms and slow down their progression.
The efficacy of several antifibrotic drugs is currently being investigated for use in the treatment of this pathology. The drugs used up to now, combined therapy of glucocorticoid-azathioprine-N-Acetylcysteine, according to recent studies are not only not effective, but are also associated with an increase in mortality and exacerbations.
In the most serious cases, as long as the patient is under 65 years of age, the only treatment option is lung transplantation, but this has many contraindications to take into account.
Oxygen
Its use will be prescribed in patients when blood oxygen levels are less than 50 mmHg or oxygen saturation less than 90%.
Although its use cannot stop the progress of the disease, it can make breathing easier and allow the patient to continue with his active life for as long as possible, in addition to reducing complications and associated problems.
Changes in lifestyle
It is essential to quit smoking and prevent other people from smoking in the presence of the patient.
Pulmonary rehabilitation
Attending pulmonary rehabilitation programs will be of great help to the affected person. They are carried out by a multidisciplinary team in which the patient will be taught everything related to his disease to help him control it effectively; you will be instructed in breathing exercises to achieve more efficient breathing; physical activity will be encouraged to increase your tolerance to physical exertion; you will be instructed in the best way to carry out activities of daily living; emotional and psychological support will be given; Their diet will be monitored... All of this aimed at delaying the evolution of the disease and improving the patient's quality of life.
Associations
AFEFPI- Association of Relatives and Patients of FPI and FPF
(Idiopathic Pulmonary Fibrosis)
Tables of Daimiel 2, local 7
Alcorcon CP 28924
Phone: 609130363
Contact: afefpi@gmail.com
Website: www.fibrosispulmonary.es
