Bronchiectasis

What happens in bronchiectasis

Healthy airways are lined with tiny hair-like cilia that sweep a thin layer of mucus upward, carrying germs and dust out of the lungs. In bronchiectasis, an initial injury such as a severe infection damages the airway walls. They lose their elasticity and become permanently widened and floppy. Mucus collects in the pouches, the cilia cannot clear it, and bacteria thrive. The body's inflammatory response damages the airways further.

Doctors call this the vicious vortex: infection, inflammation, impaired clearance and structural damage feeding into each other. Treatment aims to break the cycle at every point.

Symptoms

• a persistent cough, most days, producing phlegm (sputum) that may be clear, yellow or green
• large volumes of phlegm, often worst in the morning
• frequent chest infections (exacerbations)
• breathlessness and wheezing
• fatigue
• coughing up blood (hemoptysis), usually streaks but occasionally more
• chest discomfort
• sinus problems
• in advanced disease, clubbing of the fingertips and weight loss

Symptoms are often mistaken for repeated bronchitis, asthma or COPD for years before diagnosis.

Causes

In 30 to 40% of adults, no cause is found (idiopathic bronchiectasis). Looking for a cause still matters, because some, such as immune deficiency and ABPA, have specific treatments.

Diagnosis

• High-resolution CT (HRCT) scan: the gold standard. It shows airways wider than the blood vessels beside them and failing to taper toward the lung edges.
• Sputum cultures: identify bacteria such as Pseudomonas aeruginosa and Haemophilus influenzae, and mycobacteria.
• Lung function tests (spirometry): measure airflow obstruction.
• Blood tests: full blood count, immunoglobulin levels, tests for ABPA (total IgE and Aspergillus-specific IgE), and sometimes autoimmune and alpha-1 antitrypsin tests.
• Further tests depending on the case: sweat test or genetic testing for cystic fibrosis, nasal and ciliary tests for primary ciliary dyskinesia, and bronchoscopy.

Treatment

Airway clearance (the foundation)

Clearing mucus every day reduces infections and improves symptoms. A respiratory physical therapist will teach a routine, typically 10 to 30 minutes once or twice a day:

• active cycle of breathing techniques: breathing control, deep breaths and "huffing"
• oscillating positive expiratory pressure devices (such as flutter or Acapella devices)
• postural drainage, using gravity to help drain specific lung areas
• nebulized hypertonic saline to thin mucus, in some people

Exercise and pulmonary rehabilitation

Regular exercise helps loosen mucus and improves fitness and breathlessness. Pulmonary rehabilitation programs are recommended for people limited by breathlessness.

Treating flare-ups

Exacerbations are treated with a 14-day course of antibiotics, chosen based on previous sputum results. Many people keep a rescue pack at home and send a sputum sample before starting it.

Preventing flare-ups

For people with 3 or more exacerbations a year despite good airway clearance:

• long-term macrolide antibiotics (such as azithromycin), which reduce flare-ups
• inhaled antibiotics for chronic Pseudomonas infection
• eradication treatment when Pseudomonas is first found
• newer anti-inflammatory treatments that target neutrophil inflammation are becoming available

Treating the cause

• immunoglobulin replacement for antibody deficiency
• steroids and antifungals for ABPA
• specific CF modulator drugs for cystic fibrosis
• treatment of reflux and swallowing problems
• treatment of nontuberculous mycobacterial infection when needed

Other measures

• vaccines: yearly flu, COVID-19, and pneumococcal vaccines
• inhalers if asthma or COPD coexist
• stopping smoking
• surgery to remove a severely damaged localized area, occasionally
• lung transplant for a small number with advanced disease
• bronchial artery embolization for serious bleeding

Complications

• frequent exacerbations and hospital admissions
• coughing up blood, occasionally heavy
• chronic Pseudomonas infection, linked to worse outcomes
• respiratory failure and pulmonary hypertension in advanced disease
• anxiety and depression, which are common and treatable

Outlook

Bronchiectasis is lifelong, but most people live a normal or near-normal lifespan with good management. The outlook is worse with frequent exacerbations, chronic Pseudomonas, severe airflow obstruction and older age. Consistent airway clearance, prompt treatment of flare-ups and staying active make the biggest difference.

Frequently asked questions

What is bronchiectasis?

Bronchiectasis is a long-term condition in which the airways of the lungs are permanently widened and damaged, so mucus builds up instead of being cleared. This leads to a persistent wet cough, repeated chest infections and breathlessness.

What causes bronchiectasis?

Common causes include severe past lung infections such as pneumonia, whooping cough or tuberculosis, immune deficiencies, cystic fibrosis, primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis, rheumatoid arthritis, inflammatory bowel disease and COPD. In many adults no cause is found.

Is bronchiectasis serious?

It is a chronic condition that needs ongoing care, and frequent infections can gradually damage the lungs. However, with daily airway clearance, exercise, vaccines and prompt antibiotics for flare-ups, most people stay well and live active lives.

Is bronchiectasis contagious?

No. Bronchiectasis itself is not contagious. The bacteria that grow in damaged airways are usually not a risk to healthy people, although people with bronchiectasis or cystic fibrosis are often advised to avoid close contact with each other to prevent sharing resistant bacteria.

What is the life expectancy with bronchiectasis?

Many people with bronchiectasis have a normal or near-normal life expectancy, especially with mild disease and good management. Outlook is less favorable with very frequent flare-ups, chronic Pseudomonas infection or severe lung function loss. Your specialist can assess your individual risk.