Bronchiectasis

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It is a lung affectation in which the walls of the bronchi (airways) are abnormally extended and thickened due to inflammation and infection.

What is a biosimilar

Bronchiectasis (BQ) is one of the so-called irreversible chronic respiratory diseases affecting the lung structure.

It is a lung affectation in which the walls of the bronchi (airways) are abnormally extended and thickened due to inflammation and infection.

DICTIONARY: Bronchiectasis means “dilated airway” (“bronchus” is the name for the airways and “ectasia” means lengthening or dilation).

Infection, chronic inflammation, and exacerbations lead to a vicious circle that ends up destroying the bronchi and lung parenchyma (the tissue responsible for gas exchange, that is, the passage of oxygen from the respiratory system to the blood, to that it is distributed throughout the organism, and the elimination of carbon dioxide that is carried through the blood from all the cells of our body to the lungs to be eliminated.

Most Common Symptoms

Bronchiectasis can appear at any age, and usually do so gradually after a respiratory infection. The symptoms, which start out mild, worsen over the years.

People with BC have good periods and others with crises or exacerbations, which can last days or weeks, in which the symptoms of the disease worsen.

The most frequent symptoms and signs in people with bronchiectasis are:

Chronic cough accompanied by large amounts of thick mucus. Especially occurs in the morning and evening.
Coughing up blood (haemoptysis), because the walls of the damaged bronchi are fragile and have a greater number of blood vessels. Sometimes it is the first and only symptom.
Ringing in the chest when breathing (wheezing).
Difficulty breathing.
Fatigue or tiredness.
Fever and/or chills that occur, especially during exacerbations.
Chest pain due to increased effort to breathe.
Fingers in drumsticks or clubbing (widening of the tips of the fingers of the hands and the feet with a change in the angle formed by the nail at its birth).

Causes of bronchiectasis

Bronchiectasis can be of two types, those caused by cystic fibrosis (CF) or those not related to CF.

Those not related to CF may originate from or be related to:

Immunodeficiency: This is an alteration of the immune system that leads to the appearance and repetition of infections more frequently than usual, as well as being more serious and lasting longer. This is because the body is not able to produce the necessary antibodies to fight infections.
Chronic or recurring infections, such as tuberculosis or non-tuberculous mycobacteria (NTM).
Inflammatory bowel disease, including Crohn's disease and ulcerative colitis
Autoimmune diseases in which antibodies produced by the body attack the body's own cells, such as rheumatoid arthritis, ulcerative colitis, and Sjögren's disease
Secondary to other respiratory diseases such as:
Alpha1-antitrypsin deficiency, which is the genetic cause of COPD of the emphysema type in some people.
Chronic Obstructive Pulmonary Disease (COPD)
Asthma
Allergic bronchopulmonary aspergillosis, a type of allergic inflammation of the lungs that causes swelling of the airways.
Primary ciliary dyskinesia, a rare disease where the hair-like structures (cilia) that line our airways are damaged and do not function properly, causing mucus to not move so it can be expelled naturally.
Recurrent or chronic aspiration, in which a person inhales food, liquids, saliva, or stomach acids into the lungs.
Inhalation of substances toxic to the respiratory system such as gases, tobacco smoke...
Congenital (something people are born with).

Factors riesgo

The most common risk factors are:

Being diagnosed with CF

Having a chronic inflammatory disease.

Suffering from chronic or severe lung infections

Frequent aspirations that end up chronically damaging the lungs

Diagnosis

Tests that can help diagnose BQ are:

Blood test: to find out if the person has an infection, or the cells in charge of fighting infections in the blood have low levels or look for conditions associated with BQ.
Chest x-rays that can detect pulmonary changes caused by BQ, although they are sometimes normal.
Computed tomography (CT) with which the extent and location of lung damage will be seen.
Culture of sputum (discharge/mucus) to look at the growth of bacteria and microbes in the mucus.
Pulmonary function tests to assess the function of the lungs and their capacity. With them we can assess the ability of the lungs to retain air, to inspire (take in air) and expire (expel air) and to exchange gases. These tests do not diagnose the disease, but they help to determine the severity and see the evolution of the disease.
Bronchoscopy for the most serious cases. It consists of inserting a long, narrow tube (bronchoscope) through the airway if the cause is suspected to be an anatomical lesion or obstruction.

Treatment

There is no cure for bronchiectasis, but it can be controlled. With treatment, it is possible to lead a normal life. However, flare-ups or exacerbations need to be treated quickly to maintain the flow of oxygen to the rest of your body and prevent further damage to your lungs.

Medication

To date there are no specific drugs developed to treat or cure the disease, with which medication is administered to treat symptoms, reduce inflammation and improve the person's quality of life:

1. Fight the infection:

Antibiotics: they are the first line of attack against the bacteria that cause infections. The use of one antibiotic or another will depend on the type of bacteria. They can be administered orally, injected or nebulized.

Macrolides: They are antibiotics that, in addition to killing the bacteria, reduce inflammation of the bronchi. They can be administered for long periods of time and have side effects such as diarrhea, nausea, development of resistant bacteria...

2. Thin the mucus:

Mucolytics: They serve to thin the secretions/mucus so that it is easier to expel from the lungs. They are usually administered by nebulization.

3. Opening of the airways:

bronchodilators: They serve to open or dilate the bronchi and thus allow air to pass more easily. They can be fast-acting, whose effect is more or less immediate, or the so-called maintenance, which act continuously on the bronchi, reducing inflammation….

Respiratory physiotherapy to clean or drain the lungs

To find out which device is the right one for each person, it is advisable to consult a specialized physiotherapist.

Oxygen

As the disease progresses, most people with BQ will require oxygen as part of their treatment to improve their breathing, quality of life, and life expectancy.

This treatment will be prescribed to those people in whom their blood oxygen saturation is greatly decreased. That is, to those who have little oxygen in their blood to be able to live.

Surgery

bronchoscopy

This is a test performed with a bronchoscope, a long, thin tube with a light and camera at the end, which is inserted through the mouth or nose to reach the area of the lung where an obstruction has occurred to remove it and /or examine it, as required.

lung surgery

It is used in the most extreme cases in which there is extensive lung damage and/or severe symptoms do not disappear despite treatment. Surgery can be of several types:

Removal of a part of the lung or in its entirety when the damage is very great.
Embolization to stop bleeding in people who cough up a lot of blood.
Lung transplant in the most extreme cases.

Spanish Computerized Registry of Patients with Bronchiectasis (RIBRON)

This is a project carried out by the Spanish Society of Pneumology and Thoracic Surgery (SEPAR) through which it is intended to obtain information from patients with BQ to improve knowledge about this pathology, facilitate research and try to unify the BQ Clinical Guidelines to improve the management of this disease.

More than 40 centers with specialized units in BQ participate in this registry.

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