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Bronchiectasis

LungsLungs
Respiratory systemRespiratory system

Acquired bronchiectasis; Congenital bronchiectasis; Chronic lung disease - bronchiectasis

Definition

Bronchiectasis is a disease in which the large airways in the lungs are damaged. This causes the airways to become permanently wider.

Bronchiectasis can be present at birth or infancy or develop later in life.

Causes

Bronchiectasis is often caused by inflammation or infection of the airways that keeps coming back.

Sometimes it begins in childhood after having a severe lung infection or inhaling a foreign object. Breathing in food particles can also lead to this condition.

Other causes of bronchiectasis can include:

Cystic fibrosis, a disease that causes thick, sticky mucus to build up in the lungs
Autoimmune disorders, such as rheumatoid arthritis, Sjögren syndrome or inflammatory bowel disease
Allergic lung diseases
Leukemia and related cancers
Immune deficiency syndromes
Primary ciliary dyskinesia (another congenital disease)
Infection with non-tuberculous mycobacteria
As a complication of bronchiolitis obliterans
Asthma or chronic obstructive lung disease (uncommon)

Symptoms

Symptoms develop over time. They may occur months or years after the event that causes the bronchiectasis.

Long-term (chronic) cough with large amounts of foul smelling sputum is the main symptom of bronchiectasis. Other symptoms may include:

Breath odor
Coughing up blood (less common in children)
Fatigue
Paleness
Shortness of breath that gets worse with exercise
Weight loss
Wheezing
Low grade fever and night sweats
Clubbing of fingers (rare, depends on cause)

Exams and Tests

The health care provider will perform a physical exam. When listening to the chest with a stethoscope, the provider may hear small clicking, bubbling, wheezing, rattling, or other sounds, usually in the lower lungs.

Tests that may be done include:

Aspergillosis precipitin test (to check for signs of an allergic reaction to fungus)
Alpha-1 antitrypsin blood test
Chest x-ray
Chest CT
Sputum culture
Complete blood count (CBC)
Genetic testing, including sweat test for cystic fibrosis and tests for other diseases (like primary ciliary dyskinesia)
PPD skin test to check for a past tuberculosis infection
Serum immunoglobulin electrophoresis to measure proteins called immunoglobulins in the blood
Lung function tests to measure breathing and how well the lungs are functioning
Immune deficiency workup

Treatment

Treatment is aimed at:

Controlling infections and sputum
Relieving airway blockage
Preventing the problem from becoming worse
Treating the underlying cause (for example modulator therapy for people with cystic fibrosis)

Daily drainage to remove sputum is part of treatment. A respiratory therapist can show the person coughing exercises that will help.

Medicines are often prescribed. These include:

Antibiotics to treat infections
Bronchodilators to open up airways
Expectorants to help loosen and cough up thick sputum

Surgery to remove (resect) the lung may be needed if medicine does not work and the disease is in a small area, or if the person has a lot of bleeding in the lungs. It is more commonly considered if there is no genetic or acquired predisposition to bronchiectasis (for example, more likely to consider if there is bronchiectasis in one segment of the lung only because of prior obstruction).

In severe cases, lung transplantation might be needed.

Outlook (Prognosis)

The outlook depends on the specific cause of the disease. With treatment, most people live without major disability and the disease progresses slowly.

Possible Complications

Complications of bronchiectasis may include:

Cor pulmonale
Coughing up blood
Low oxygen levels (in severe cases)
Recurrent pneumonia
Depression

When to Contact a Medical Professional

Contact your provider if:

Chest pain or shortness of breath gets worse
There is a change in the color or amount of phlegm you cough up, or if it is bloody
Other symptoms get worse or do not improve with treatment

Prevention

You can reduce your risk by promptly treating lung infections.

Childhood vaccines a yearly flu vaccine, and COVID-19 vaccines help reduce the chance of some infections. Avoiding upper respiratory infections, smoking, and pollution may also reduce your risk of getting this infection.

References

Chang AB, Redding GJ. Bronchiectasis and chronic suppurative lung disease. In: Wilmott RW, Deterding R, Li A, et al, eds. Kendig's Disorders of the Respiratory Tract in Children. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 26.

O'Donnell AE. Bronchiectasis, atelectasis, cysts, and localized lung disorders. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 84.

Solomon GM, Chan ED. Bronchiectasis. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 69.

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Review Date: 7/31/2022

Reviewed By: Denis Hadjiliadis, MD, MHS, Paul F. Harron, Jr. Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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