Bronchiectasis: symptoms, causes, treatment

Medically reviewed: 8, January 2024

Bronchiectasis: Definition of the disease

Bronchiectasis is a disease of the respiratory tract in which persistent dilatation of the lumen of the bronchi occurs (bronchiectasis). This process is associated with impaired elasticity and destruction of the walls of the bronchi, it is accompanied by inflammation, impaired blood supply, the germination of coarse scar tissue, and may be a consequence of underdeveloped branches of the bronchial tree.

What is bronchiectasis?

Bronchiectasis is the dilation and destruction of large bronchi caused by inflammation and chronic infection.
Purulent inflammation of the wall of the respiratory tract leads to further irreversible expansion of the walls of the bronchi. In this case, their drainage function is disrupted, i.e., accumulation and prolonged presence of purulent viscous sputum in the bronchi occurs. It forms plugs and fills the final sections of the respiratory tree. This, in turn, leads to the appearance of airless areas of the lungs (atelectasis), areas of increased swelling of the lungs (emphysema), as well as areas of cirrhosis – cicatricial transformation of normal lung tissue.

Bronchiectasis occurs in 2-4% of the adult population. Its detection in 2/3 of patients occurs before the age of 20 years. In children, the disease is detected equally often in boys and girls, and among adults, men are affected 1.5-3 times more often than women. In recent years, this diagnosis has been made less and less frequently, despite the improved diagnostic capabilities of modern medicine. This may be due to more effective treatment of respiratory diseases in childhood, including diseases that cause bronchodilation.

Causes of the bronchiectasis

The main reason for the development of bronchiectasis in childhood and adolescence is inflammation of the end sections of the bronchi (bronchioles).

The disease can develop as a result of a respiratory infection – with untreated pneumonia, after measles, whooping cough, or adenoviral infection. Tuberculous and non-tuberculous mycobacteria can also cause bronchiectasis.

In addition, other factors can lead to dilation of the bronchi.

Errors in the treatment of acute inflammatory processes in the lungs resulting from local disturbances in the structure of the bronchi.

Genetic factors

Bronchiectasis can be caused by hereditary structural abnormalities of the lungs associated with weakness of the bronchial wall:

• Siewert-Kartagener syndrome – structural and functional disorders of the ciliated epithelium of the bronchi;
• Mounier-Kuhn syndrome is a congenital elongation and expansion of the cartilaginous rings of the trachea and large bronchi;
• Williams-Campbell syndrome – congenital failure of bronchial cartilage;
• congenital disorders of connective tissue structure (Marfan syndrome);
• congenital absence/underdevelopment of part of the lung.

A severe hereditary disease – cystic fibrosis. Widespread bronchiectasis is an integral part of the symptoms of this disease. Disruption of the epithelial cells lining the respiratory tract leads to structural disorders of the bronchi. This is usually a two-way common process. In such patients, bronchiectasis is accompanied by chronic inflammation of the sinuses and pancreas. In most cases, symptoms of bronchiectasis begin in early childhood; less often, the diagnosis is made in adulthood due to the erased clinical picture.

Immune disorders (impaired production of immune response cells and immunoglobulins) can also cause the hereditary nature of bronchiectasis.

Inhalation (aspiration) of foreign bodies, nasopharyngeal secretions, toxic and chemical damage to the lungs.

Can bronchiectasis be cured?

Bronchiectasis refers to chronic diseases with irreversible structural changes in the bronchial tree.

Symptoms of bronchiectasis

The disease is long-term and chronic. There is a wave-like course of the disease with periods of exacerbation (worsening of symptoms) and remission (absence and/or a small number of manifestations of the disease). One- or two-sided arrangement of dilated bronchi is possible; more often, changes are located in the lower parts of the lungs. Localization of bronchiectasis is important both for finding the cause of the disease and for its treatment.

Cough

The most common symptom of bronchiectasis is a chronic cough producing sputum. Rarely, this symptom may be absent (dry bronchiectasis). Sputum disappears more often in the morning. During the period of remission, its amount usually does not exceed 50 ml per day. During an exacerbation, sputum acquires an unpleasant odor and is discharged abundantly throughout the day (300-500 ml per day).

Chest pain

During exacerbations, chest pain may appear. This is due to pleural involvement. The pain may intensify with a deep breath, but sometimes has no clear localization and is accompanied by a feeling of compression, distension, and difficulty breathing. Often during exacerbations, body temperature rises, the fever is prolonged, combined with chills, sweating, weakness, and increased fatigue.

Shortness of breath and wheezing

With a widespread process and the addition of bronchial obstruction, patients may complain of mixed shortness of breath and wheezing.

Decreased ability to work

Often, patients are concerned about a decrease in exercise tolerance, and respiratory failure develops—a decrease in the ability of the lungs to maintain the required concentration of oxygen in the blood.

Fingers of Hippocrates

The symptom of “Hippocrates’ fingers” or “drumsticks” is observed during a long-term course of the disease with frequent exacerbations. It is characterized by flask-shaped thickening of the terminal phalanges of the fingers. Also, with a long course of the disease, weight loss is possible.

Developmental delay

Developmental delay is not typical for bronchiectasis; it occurs in rare cases with congenital anomalies of the bronchi.

The main reason for visiting a doctor may be a suspicion of recurrent bronchitis or bronchial asthma due to shortness of breath and cough. Bronchiectasis can also be suspected by relapses of community-acquired pneumonia and episodes of hemoptysis.

Pathogenesis of bronchiectasis

Destruction of the structure of the muscle layer and elastic fibers of the wall of the respiratory tract is accompanied by their expansion and disruption of the function of air conduction. Stagnation of mucus causes a hacking cough with stretching of the walls, spread of sputum, and blockage of the terminal sections. A decrease in the airiness of individual sections of the bronchi leads to compaction of lung tissue, which, in turn, reduces the efficiency of blood supply. The formation of bronchiectasis is often associated with the development of foci of increased airiness of the lungs and the spread of chronic inflammatory changes in the bronchi.

A chronic inflammatory process involving bacteria reduces the elastic properties of the bronchial wall; it stretches and protrudes, causing it to become thinner. Scar changes in the interstitial (connective) tissue develop around the lungs, which leads to additional stretching of the lumen of the airways. Protrusion of the wall is carried out through local damage.

It is also important to reduce the protective properties of the bronchial tree against microorganisms, as a result of which biofilms containing an infectious agent (Pseudomonas aeruginosa, etc.) are formed on the surface of the respiratory epithelium.

Classification and stages of development of bronchiectasis

Bronchiectasis is classified according to its origin, prevalence, nature of lung deformation and other characteristics.

By origin:

• primary (congenital), associated with hereditarily determined changes in the structure and function of the airways;
• secondary (acquired), arising as a result of repeated inflammatory processes in the lungs and bronchi.

According to the clinical course:

• remission phase;
• exacerbation phase.

By prevalence:

• unilateral process (damage to a segment, lobe of the lung);
• two-way process.

When using instrumental research methods, such as computed tomography, bronchoscopy, it is possible to determine the nature of the distribution of bronchiectasis:

• central (changes in large airways);
• peripheral (damage to small bronchi from the 5th to 16th branches of the tracheobronchial tree).

According to the nature of lung deformation:

• saccular;
• cylindrical;
• cyst-like;
• fusiform;
• mixed.

Forms of bronchiectasis

According to the severity of the disease:

• mild form – 1-2 exacerbations per year, long-term remissions, during which the patients’ performance is not impaired;
• moderate – exacerbations are observed more than twice a year, sputum production is 50-100 ml per day, in the remission phase there is a wet cough, moderate impairment of respiratory function and decreased performance;
• severe – exacerbations are frequent and prolonged, sputum production is more than 200 ml per day, the remission phase is short-term, accompanied by disability.

Post-infectious:

• lower respiratory tract infections in childhood;
• pneumonia, accompanied by destruction of lung tissue in adults;
• tuberculosis and non-tuberculous mycobacteriosis;
• measles, whooping cough, adenovirus infection.

Obstructive:

• blockage of the bronchi by a foreign body;
• external compression of the airways.

Inhalation damage:

• inhalation of toxins and irritating gases, smoke;
• temperature damage;

Aspiration:

• penetration into the respiratory tract of an irritating secretion infected with bacteria, which is possible with gastroesophageal reflux,
• aspiration of secretions from the upper respiratory tract and gastric contents with the development of pneumonia, during the procedure of sanitation (cleansing of mucus) of the respiratory tract;

Chronic diffuse lung diseases:

• idiopathic pulmonary fibrosis;
• sarcoidosis;
• connective tissue diseases affecting the lungs.

Idiopathic inflammatory disorders (for diseases of unknown etiology):

• inflammatory bowel diseases;
• recurrent multiple lesions of cartilage tissue;
• ankylosing spondylitis with the development of inflammation and impaired mobility of intervertebral joints

Stages of the bronchiectasis

1. The first stage is limited changes in the small bronchi with their filling with mucus.
2. The second stage is inflammation and suppuration with a violation of the integrity of the layer of cells lining the lumen of the bronchi from the inside, the development of scar tissue in the thickness of the bronchi.
3. The third stage – the dilated bronchi are filled with pus, the process progresses with the release of lung tissue into the surrounding bronchi, where scar tissue develops. Chronic suppurative intoxication leads to disruption of the nutrition of the heart muscle and other internal organs with disruption of their function.

Complications of bronchiectasis

The long-term nature of the disease with frequent exacerbations contributes to the development of complications.

Pulmonary hemorrhages

In 10-15% of cases, streaks of blood may appear in the sputum (hemoptysis), and episodes of pulmonary hemorrhage occur less frequently.

Spontaneous pneumotrax

Symptoms of bronchiectasis may be accompanied by a picture of a sudden rupture of lung tissue with the appearance of air in the pleural cavity (spontaneous pneumothorax) – 0.7% of cases.

This condition is manifested by sharp, intense (“dagger-like”) pain in the chest on the side of the rupture, the sudden appearance and intensification of shortness of breath that occurs when inhaling. At this time, the cough intensifies, with the accumulation and increase in the amount of air in the pleural cavity, blood pressure decreases and the pulse increases. The patient may lose consciousness due to compression of the heart and large vessels located in the space between the lungs (mediastinum) by free air.

Chronic respiratory failure

Chronic respiratory failure (CRF) is manifested by shortness of breath, decreased exercise tolerance, and is characterized by an increase in respiratory rate. The criterion for CDN is a decrease in blood saturation (oxygen saturation) to 95% or lower.

Pneumosclerosis

Pneumosclerosis may not manifest itself clinically during a local process; when the disease spreads, manifestations of respiratory failure are observed.

Pulmonary heart

“Cor pulmonale” is an enlargement and expansion of the right chambers of the heart, which has developed as a result of lung diseases. Initially, cor pulmonale may be asymptomatic, although patients usually experience severe manifestations of the underlying disease (eg, shortness of breath). The first manifestation of cor pulmonale is a rapid heartbeat.

Absedation

Among the complications of bronchiectasis there are also suppurative processes spreading to the lung tissue and pleura:

• lung abscess (1.8%),
• empyema (0.4%).

Abscess formation is the formation of a large purulent cavity in the lungs. When an abscess forms, there is a persistent increase in body temperature, and when it opens, a profuse discharge of purulent sputum (“a mouth full”) is noted in the bronchi. This complication often requires surgical treatment.

Empyema of the pleura

Pleural empyema is the appearance of free pus in the pleural cavity, accompanied by severe intoxication and chest pain on the affected side. This condition, along with lung abscess, is life-threatening and requires active drainage (removal of pus) and surgical supervision.

Septicemia (blood poisoning)

The above complications may be accompanied by the development of sepsis – the appearance of microbes in the blood with the development of multiple organ failure.

Amyloidosis

Extrapulmonary complications of long-term bronchiectasis include amyloidosis. This is a pathological process of production and accumulation of amyloid protein in the tissues of internal organs. Its high concentration leads to dysfunction and damage to the structure of these organs. Also a complication may be the formation of ulcers and erosions on the mucous membrane of the stomach and duodenum.

Diagnosis of bronchiectasis

The purpose of diagnosing bronchiectasis is to establish the pathological process that caused the development of structural changes in the bronchi, the nature of the changes, their localization and prevalence. In the diagnosis, it is necessary to take into account characteristic signs: symptoms of cough with sputum (sometimes with streaks of blood in it), shortness of breath, decreased exercise tolerance. You should always pay attention to episodes of repeated inflammatory diseases of the lungs and bronchi previously suffered, the age of onset of the disease, and extrapulmonary symptoms.

Consultation with an ENT doctor

If symptoms of the disease are detected, you should contact a therapist or pulmonologist. In some cases, there are symptoms of chronic nasal congestion, as a manifestation of chronic sinusitis. If these symptoms are present, additional consultation with an ENT doctor is necessary. A routine examination of patients with bronchiectasis by an ENT doctor is carried out once a year.

Patient examination

During an external examination of the patient, attention is drawn to thickening of the terminal phalanges like “drumsticks” and deformation of the nails (“watch glasses”) during a long process.

These changes occur due to the stimulating effect of low oxygen levels in the blood on the growth of the periosteum. A careful examination of the chest can reveal its asymmetry and deformation: a decrease in the size of its part with local compaction of the lung with the development of scar tissue; barrel-shaped swelling with anterior-posterior expansion with increased airiness (emphysema).

Listening to the lungs

When listening to the lung using a phonendoscope (auscultation), foci of moist rales of different calibers are determined depending on the diameter of the bronchi that have undergone changes. Breathing can be harsh (exhalation is heard throughout its entire length) or weakened (silent exhalation).

Laboratory research

The goal of diagnostics is to determine how severe the inflammation is – determining C-reactive protein, the number of leukocytes, platelets, and ESR in the blood. With a long course of the disease, anemia develops with a decrease in the number of red blood cells and hemoglobin.

A general analysis of sputum also reveals signs of inflammation: an increase in the number of leukocytes, red blood cells can be detected when capillaries are destroyed in the cavities of the dilated bronchi, bacteria, and fungi. Sputum culture identifies pathogenic pathogens.

A general blood test, urine test and biochemical blood test are examined once every 6 months and during exacerbations of the disease. It is necessary to pay special attention to the level of red blood cells, hemoglobin (secondary erythrocytosis), the level of leukocytes and the nature of the leukocyte formula, the level of C-reactive protein.

Proteinuria and specific gravity in urine should be monitored (a manifestation of secondary renal amyloidosis).

Instrumental research methods

In making a diagnosis, instrumental research methods are of key importance, among which the leading one is computed tomography of the lungs.

A clear picture of the prevalence, form and nature of bronchiectasis is recorded using spiral computed tomography. It allows you to determine the unevenness of the lumen of the bronchi, the absence of narrowing of the bronchi from the center to the periphery (the “tram track symptom”), the presence of secretions in their lumens, the expansion of the bronchus with the preserved diameter of the lumen of the underlying vessel (the “ring sign”).

Concomitant signs of decreased and increased pulmonary ventilation, scar changes, and the formation of complications in the form of an abscess can also be detected with this type of imaging.

X-rays of light

Survey radiography and digital fluorography reveal heaviness, cellularity, cystic deformation of the lung pattern, areas of compaction and increased airiness of the lung tissue.

Bronchoscopy

Bronchoscopic examination serves as a good complement to computed tomography in determining bronchiectasis. During this procedure, it is possible to take bronchial swabs and biopsy material from the mucous membrane, if necessary. In addition, bronchoscopy is used for therapeutic purposes.

Functional tests

A study of pulmonary function with a bronchodilation test (a study of bronchial relaxation) is used to prescribe and adjust inhalation therapy. Tests are most informative when carried out in the morning (before 11:00), carried out on an empty stomach or after a light breakfast. On the eve of the study, it is necessary to abandon inhalation therapy.

Bronchography

Bronchography is currently not used to diagnose bronchiectasis due to its invasiveness (deep penetration through the tracheobronchial system) and poor tolerability.

Electrocardiography

An electrocardiogram is performed once a year to evaluate possible rhythm disturbances, thickening of the right heart with the development of pulmonary hypertension and cor pulmonale.

Treatment of bronchiectasis

The treatment of bronchiectasis is based on the principle of timely anti-inflammatory and drainage therapy, which is aimed at evacuating secretions from the dilated bronchi. The minimal invasiveness of the procedures is also important, since each episode of penetration of foreign objects (including endoscopic tubes) into the lungs increases the risk of additional infection of damaged areas of the bronchi.

Drug treatment for bronchiectasis

Currently, the safest and most effective is the inhalation form of delivering medicinal solutions to the lungs. For this purpose, nebulizers are used – devices that spray drug solutions throughout the tracheobronchial tree.

With their help, both at home and in medical institutions, mucolytics are inhaled – drugs that dilute sputum and stimulate the work of the ciliated epithelium that lines the lumens of the bronchi (for example, ambroxol solution).

For anti-inflammatory purposes, inhalation of budesonide suspension is used to treat bronchiectasis; a compressor nebulizer is suitable for it. This drug is inhaled in courses, especially in the presence of bronchial obstruction (narrowing of the lumen of the bronchi) and associated shortness of breath with difficulty exhaling.

Expansion of the lumens of the bronchi is also achieved by inhalation of beta-2-agonists and M-anticholinergics and their combinations (salbutamol, ipratropium bromide, fenoterol).

There are other inhaled drugs that do not require the use of a nebulizer: tiotropium bromide, umeclidinium bromide, formoterol, salmeterol, indacaterol, vilanterol. They are used in the form of powder, liquid inhalers, and aerosols. An important aspect is strict adherence to the inhalation technique, so you must bring the device prescribed for treatment to each doctor’s appointment, which will allow you to check the process and correct errors in a timely manner.

During exacerbations of bronchiectasis, the use of antibacterial drugs is indicated. Their use depends on the results of sputum cultures, as well as the timing and drugs of previous antibiotic therapy. Typically, beta-lactams (protected semi-synthetic penicillins, cephalosporins), drugs with antipseudomonal activity (fluoroquinolones, 3-4 generation cephalosporins, aminoglycosides) are used to treat bronchiectasis.

Instrumental methods

In the case of a severe form of the disease with frequent exacerbations, it is sometimes justified to conduct a series of sanitation bronchoscopy performed under local anesthesia. This will allow you to cope with exacerbations more quickly. The procedure must be performed in a hospital setting, less often on an outpatient basis.

Surgery for bronchiectasis

Surgical treatment methods are indicated for local processes in the following cases:

• the local process is accompanied by pulmonary hemorrhages;
• within two to three years it is not possible to achieve stable remission;
• development of local cirrhosis;
• pulmonary bleeding more than 200 ml/day cannot be treated with drugs.

Surgery is possible at any age. Operations for bilateral resection (removal of part of the lung) are usually performed at intervals of 6-8 months.

Maintaining proper nutrition

In the treatment of patients with bronchiectasis, along with other methods, therapeutic nutrition is of great importance. The diet should contain an increased amount of protein (up to 160 g), moderate amounts of fat and carbohydrates.

It is necessary to include vitamins A, B1, B2 and C in the diet. Vitamin A helps improve the regeneration of the mucous membrane of the respiratory tract, vitamins B1, B2 and C improve oxidative processes and protein metabolism. Salt content is limited. Meals should be at least five times a day. Diet No. 5 with a high content of protein and vitamins is recommended.

Oxygen therapy for bronchiectasis

In the presence of chronic respiratory failure with a decrease in blood saturation to 89% or less, long-term oxygen therapy at home using oxygen concentrators is recommended. Also, situational oxygen therapy is carried out in a hospital setting at the stage of treatment of exacerbations of the disease, accompanied by a decrease in saturation to 95% or less.

Physiotherapy

During the period of exacerbation of the process, electrophoresis and UHF therapy are used on the chest – a low-thermal dose lasting 10-15 minutes. During the period of remission, general UV irradiation and inhalation of mucolytic agents are carried out. To improve the functional state of the lungs and the drainage function of the bronchi, chest massage is used. Vibration massage is carried out using a special vest or with direct percussion on the chest in a lying position in the direction from bottom to top. It is better to carry it out after the inhalation procedure in order to increase the rate of disintegration (breakdown) of sputum and its removal from the airways.

In case of illness, evacuation of bronchial secretions is important. To do this, static and dynamic postural drainage is used – adopting a body position in which coughing up sputum is most effective.

Exercise therapy

Therapeutic exercise should be carried out by a patient with bronchiectasis constantly. Exercises are used to increase the movement of the diaphragm and intercostal muscles in combination with postural drainage of the bronchi (body position for better sputum discharge depends on the location of bronchiectasis). Breathing exercises increase the pressure created in the lumen of the bronchi, promote their cleansing and more uniform filling with air during inhalation.

Gymnastics can be done independently or with the connection of portable exercise machines, some of which can create additional vibration.

Contraindications for physical therapy:

• pulmonary hemorrhage; stage III cardiovascular failure;
• an increase in temperature to 38-39 ° C, not due to the accumulation of sputum.

If there is sputum, therapeutic exercises begin with exercises that promote the removal of sputum:

• postural drainage is used;
• drainage exercises and their combination.

When a large amount of sputum is produced, exercises that drain the bronchi are performed up to 8-10 times a day:

• in the morning before breakfast for 20-25 minutes,
• 2 hours after breakfast, lunch, every hour before dinner and an hour before bedtime.

Forecast. Prevention of bronchiectasis

Prevention of secondary (acquired) bronchiectasis consists of timely prevention and effective treatment of infectious inflammatory diseases of the lungs. For this purpose, vaccination against pneumococcal infection, measles, and whooping cough seems rational.

The pneumococcal vaccine is also an important tool for secondary prevention in the presence of bronchiectasis. It helps reduce the frequency of exacerbations and prevent complications of diseases. To prevent exacerbations, it is recommended to harden yourself and engage in physical therapy.

In connection with the developed therapeutic measures to sanitize the tracheobronchial tree (eliminate mucus from the affected bronchi) and prevent exacerbations, the prognosis for bronchiectasis has improved in recent years.

Many patients live to old and senile age, but the quality of life of patients suffers due to increasing cardiopulmonary failure. The development of chronic pulmonary heart disease can lead to permanent disability. After surgical treatment, recovery occurs in more than 75% of patients, the remaining 25% show a significant improvement in their condition.

 

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