An Overview of Bronchiectasis

A Type of COPD with Childhood Causes

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

Bronchiectasis is defined as a form of chronic obstructive pulmonary disease (COPD) in which the large airways of the lungs become damaged and widened. It is common in people with cystic fibrosis, but may also occur in people who experience repeated respiratory infections in childhood. The main and hallmark symptom of the condition is a cough that is productive of sputum (a wet cough). Though the condition may be suspected on a chest X-ray, a chest CT is usually needed to make the diagnosis, and lung function studies are often used to determine the severity. The incidence of bronchiectasis is currently increasing in the United States.

The most important goal of treatment is to identify and address the underlying cause. Symptomatic treatment also includes reducing infections, lowering inflammation, and keeping the airways healthy and moist. Both the severity and prognosis of bronchiectasis vary widely, and depend to a large degree on the cause of the disease. While regular medical care and follow-up are needed, many people are able to live fully with the condition, and some have even climbed mountains.

We will look at the natural history, symptoms, and treatment of this disease, and then share some tips on coping with the stigma and frustration of coping with this condition.

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 ripe for infections to begin, setting up a sometimes vicious cycle.

When mucous collects in the dilated airways, bacteria can grow and cause recurrent lung infections. Infections then lead to exacerbations of the disease and further widening and scarring of the bronchi. Though this sounds ominous, working carefully with your doctor through regular check-ups, and staying on top of immunizations allows many people to live active lives despite their disease.

Incidence

Though bronchiectasis is relatively common, affecting between 350,000 and 500,000 adults in the United States, people are often less familiar with this type of COPD that they are with emphysema or chronic bronchitis. This feeling of being alone can create a lot of anxiety, and support is extremely important. Added to this is the stigma of COPD being a "smoker's disease," when bronchiectasis is most commonly caused by childhood infections or genetic conditions. The condition is more common in women, and this is thought to be related to greater genetic susceptibility to the processes that lead to the disease.

Symptoms of Bronchiectasis

The signs and symptoms of bronchiectasis are usually related 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, and is sometimes foul-smelling. This overproduction of mucus is a hallmark of the disease.
  • Symptoms due to repeated infections such as a fever and respiratory symptoms.

Other symptoms are less common, and usually only present when the condition has been ongoing for many years. This may include:

  • 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 the disease is fairly advanced.
  • Unintentional weight loss. A loss of five percent or more of body weight over a period of six months may occur, but should always be investigated for other causes.
  • 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.

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 may 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 immune disorders or HIV, due to an allergic reaction to a fungus known as allergic bronchopulmonary aspergillosis, atypical mycobacterial infections, or due to primary ciliary dysplasia.

Bronchiectasis may be localized to one area of a lung, or generalized throughout both lungs.. When it is localized (in one area) or all over (diffuse) often depends on the underlying cause.

Different medical conditions often affect the airways at different levels (though there is overlap) as well:

  • Upper airways: Tuberculosis, cystic fibrosis
  • Middle airways: Atypical mycobacterial infections
  • Lower airways: Recurrent childhood infections (post-infective), immunodeficiencies
  • Central airways: Bronchopulmonary aspergillosis, cystic fibrosis

Diagnosis of Bronchiectasis

diagnosis of bronchiectasis begins with a careful history and physical exam followed by imaging and lung function studies.

A chest X-ray can sometimes give clues to the presence of bronchiectasis, though these clues, even when present, are often mild and may easily be missed. The classic finding is referred to as the "tram-track" sign, in which the airways have the appearance of a railroad track due to the dilated airways. Other signs associated with diagnosis include a broncoalveolar ratio greater than one, and a lack of tapering of airways.

The most helpful test by far is a high resolution chest CT scan. Once the diagnosis is likely, further testing is done to evaluate the severity and look for underlying causes.

Testing Severity

Pulmonary function tests can help determine the severity, and usually show decreased lung capacity (a reduced FVC) and a decreased FEV1, especially with exacerbations.

Tests for Causation

Sometimes the cause will be clear, such as when a person has a known diagnosis of cystic fibrosis, or had repeated infections (especially with infections such as whooping cough) in childhood. Possible tests may include:

  • Bronchoscopy: A bronchoscopy may be done to rule out a foreign body or lung cancer.
  • Gene testing for cystic fibrosis: With mild variants of cystic fibrosis, the condition occasionally goes undiagnosed until a person is in their 50s.
  • Testing for tuberculosis
  • CBC, Serum IgE, and skin testing for aspergillosis
  • Serum IgA, IgM, and IgG (should be done for all people with the diagnosis), and serum electrophoresis if elevated
  • Next generation sequencing: A 2018 study found a number of rare genetic variants that may affect mucus or cause ciliary defects, and when the diagnosis is uncertain, genetic screening may be helpful in determining the underlying causes.
  • Sputum cultures for atypical mycobacteria
  • Testing for rheumatoid factor, ANA, etc. if signs of autoimmune disease are present

Differential Diagnosis

Other diagnostic tests may be done to rule out other conditions that have symptoms similar to bronchiectasis. Some of these include:

  • Alpha-1-antitrypsin deficiency
  • Tuberculosis
  • Asthma
  • Pneumonia
  • Gastroesophageal reflux disease (GERD)
  • Pleural effusion
  • Chronic sinusitis

Treatments

The most important step the treatment of bronchiectasis is to treat the underlying cause of the disease, as the changes in the airways are, by definition, irreversible.

Infections are common and need to be treated and monitored. Research is in progress looking at the possibility of using inhaled antibiotics to reduce the risk of resistance with non-cystic fibrosis-related bronchiectasis. Mucus thinning medications may be used for some people.

Airway Clearance Techniques

Airway clearance techniques may help with decreasing sputum and with lung function. Pulmonary rehabilitation with a respiratory physiotherapist who is knowledgeable about bronchiectasis is helpful.

Oxygen therapy may be needed if the disease is severe, and occasionally surgery is needed to remove a very damaged area of a bronchus. Lung reduction surgery may be used to removed areas of diseased lung in which infections persist. Lung transplantation may be considered for those who have progressing disease that isn't responding to therapy.

Cystic Fibrosis vs. Non-Cystic Fibrosis Associated Bronchiectasis

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.

Complications

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

It's now known that COPD is a risk factor for lung cancer even in people who have never smoked, so it's important to look for potential secondary causes when there is a worsening of symptoms.

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.

Coping with the Stigma

Just as the general public is learning that lung cancer can and does occur in never smokers, in time the public may become more educated about bronchiectasis. Until then, everyone living with the disease can do their part to teach others. That it's not related to smoking, but even if it was, everyone deserves caring and compassion and the best medical care possible.

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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