An Overview of Bronchiectasis

A Type of COPD with Childhood Causes

female doctor listening with a stethoscope to a woman's chest
What is bronchiectasis and what are the causes and symptoms?. Istockphoto.com/Stock Photo©AlexRaths

If you or a loved one have been told you have bronchiectasis, you're probably trying to understand this condition. Though it is relatively common, people are usually less familiar with this type of COPD than emphysema or chronic bronchitis. What are the symptoms, causes and risk factors, and treatments for this condition?

Definition: Bronchiectasis

Bronchiectasis is a form of chronic obstructive pulmonary disease (COPD) in which the large airways of the lungs (bronchi) become damaged and widened. Mucous can collect in these dilated airways, allowing bacteria to grow and cause recurrent lung infections. The disease may be localized to one area of a lung, or generalized throughout both lungs.

Natural History of Bronchiectasis

Bronchiectasis often begins with repeated respiratory infections in childhood. These infections result in a thickening, widening, and scarring of the bronchi, the tubes which pass from the trachea and down into the alveoli, the area in the lungs where the exchange of oxygen and carbon dioxide takes place. Meanwhile, the accumulation of mucus in the widened areas makes conditions right for infections to begin.  Infections lead to exacerbations of the disease and further widening and scarring of the bronchi.

Causes and Risk Factors

Bronchiectasis is often caused by repeated lung infections during childhood. Common infections that could lead to bronchiectasis such as whooping cough are less common due to immunizations, but yet the incidence of bronchiectasis continues to rise.

Roughly 50 percent of cases are due to the hereditary condition cystic fibrosis. It can also occur due to obstruction of the airways by a tumor such as lung cancer, or a foreign body, due to immune suppression from either congenital disorders or HIV, due to an allergic reaction to a fungus known as allergic bronchopulmonary aspergillosis, or due to primary ciliary dysplasia.

Symptoms of Bronchiectasis

The signs and symptoms of bronchiectasis of bronchiectasis most often occur due to the widening of airways and the accumulation of mucus that sets the stage for infection. Common symptoms include:

  • persistent cough: The cough associated with bronchiectais occurs daily and is often worst in the early morning and then again late at night.
  • Mucus production: The daily cough of bronchiectasis is accompanied by mucus which can be clear, yellow, green, or even blood tinged. This overproduction of mucus, that can be foul-smelling at times, is a hallmark of the disease.
  • Symptoms due to repeated infections such as a fever and respiratory symptoms.
  • Wheezing.
  • Coughing up blood.
  • Pain with a deep breath (pleuritic chest pain).
  • Clubbing: Clubbing is a condition in which the fingertips take on the appearance of an inverted spoon.
  • Shortness of breath: Shortness of breath often occurs when
  • Unintentional weight loss.
  • Fatigue: The fatigue associated with bronchiectasis can be profound, and is often associated with both the effort that goes along with coughing, and the decreased exchange of oxygen in the lungs.

Diagnosis of Bronchiectasis

diagnosis of bronchiectasis begins with a careful history and physical exam followed by imaging and lung function studies. Some tests that aid in the diagnosis include:

  • Chest x-ray: A chest x-ray may show some signs of the disease, but other tests (such as a CT) are usually needed to confirm or refute the diagnosis.
  • Chest CT scan: The diagnosis is usually made based on the appearance of a CT scan.
  • Pulmonary function tests can help with the diagnosis as well as the severity.
  • A bronchoscopy may be done to rule out a foreign body or lung cancer.

Other diagnostic tests may be done to rule out other conditions (such as tuberculosis, cystic fibrosis, and more) that can have similar symptoms.

Treatments

The most important step the treatment of bronchiectasis is to treat the underlying cause of the disease. Infections are common and need to be treated and monitored. It was thought that treatments used for cystic fibrosis could help for people without cystic fibrosis with bronchiectasis, but more recently it appears that some of these treatments may even be harmful. Airway clearance techniques may help with decreasing sputum and with lung function. Oxygen therapy may be needed if the disease is severe, and occasionally surgery is needed to remove a very damaged area of a bronchus.

Complications

In addition to repeated infections, some people develop atelectasis - a collapse of part of or all of a lung and respiratory failure may occur. The disease can also lead to right heart failure, something known as cor pulmonale.

Prognosis

The prognosis of bronchiectasis depends on the underlying cause. For some people, this can be very serious or fatal (as in the case of cystic fibrosis) whereas for others, living a full and mostly normal life is possible.

Factors associated with longer life expectancy include a high body mass index (in other words, not being underweight), regular vaccinations, especially against influenza and pneumonoccus, and regular clinic visits.

Factors associated with a poorer prognosis include hypoxia (a low blood oxygen level), hypercapnia (an elevated blood carbon dioxide concentration), increased shortness of breath, and more severe disease as seen on radiology studies.

Example: Joshua developed bronchiectasis when his lung tumor obstructed one of the large airways in his lungs.

A Word From Verywell

Bronchiectasis, unlike other forms of COPD, is often related to infections early in life (rather than lifestyle measures such as smoking later on). Yet despite advances in immunizations for infections, the condition is increasing in the United States. As we learn more about the disease, we are finding that it is very heterogenous, meaning that the characteristics of the disease and underlying mechanisms can vary considerably from person to person. What this means for people living with the condition is that there is not a one-size-fits-all approach to management, but each person with bronchiectasis needs to be considered as an individual to determine the best treatment options.

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