A recently published review article led by researchers from the Alkmaar Medical Center in the Netherlands outlines the epidemiology, clinical presentation, diagnostic workup, and treatment options in adult non-cystic fibrosis (non-CF) bronchiectasis.  The study, entitled, “Non-cystic fibrosis bronchiectasis: clinical presentation, diagnosis and treatment, illustrated by data from a Dutch Teaching Hospital,” was published in The Netherlands Journal of Medicine and aimed to increase awareness of the often underdiagnosed condition and provide practical tools for diagnosis and treatment.

About Bronchiectasis

According to the American Lung Association, bronchiectasis is a condition involving abnormal widening of the bronchial tubes and the formation of small pockets of infection. Bronchiectasis typically occurs as a complication of primary infections such as bronchitis, pneumonia, pertussis (whooping cough) or tuberculosis. Bronchiectasis can result in vicious cycle of excessive bronchial inflammation, bacterial colonization and infection, for patients diagnosed with the condition. It can develop at any age. It begins most often in childhood, but symptoms may not appear until much later.

This Study

For this review study, the researchers analyzed the current literature on bronchiectasis and conducted a medical chart review of 236 adult patients with recurrent lower respiratory tract infections and high-resolution CT-proven non-CF bronchiectasis, who visited the outpatient clinic for respiratory diseases of a large Dutch teaching hospital between 2000 and 2010.

The review results were divided into sections that highlighted the history of the condition, epidemiology, and reasons why it is often an underdiagnosed condition.

History

According to the literature, bronchiectasis was common in the Western world around World War I, and it carried a poor prognosis in which over 40% of all patients died of respiratory causes before the age of 40.  Improved socio-economic status, successful nationwide vaccination programs for whooping cough and measles, and, most importantly, the availability of antibiotics reduced both the morbidity and mortality rates in developed countries.  In which case, bronchiectasis became an “orphan disease,” as a result of which the focus of clinicians and researchers diverted away from this condition, which was now considered rare, and no longer a condition of high priority. Even with adequate antibiotic treatment, however, bronchiectasis still has the potential to cause substantial morbidity, including repeated lower respiratory infections complicated with a disabling productive cough and shortness of breath, all of which significantly impact a patient’s quality of life.

Important developments in the last decade include the introduction of international guidelines, the proposal for a validated scoring system for disease severity and the first large randomized trial on antibiotic maintenance treatment for those patients with frequent exacerbations.

Epidemiology

Patients with bronchiectasis were found to spend more days in hospital and have higher annual medical care expenditure as compared with matched controls. Three recent epidemiological studies show a high incidence of bronchiectasis among New Zealand’s and Australia’s indigenous population and inhabitants of remote areas in Alaska. In the developed world, estimated prevalence ranges from 0.42 per 100,000 in 18-34 year olds to 272 per 10,000 in those over 75.5 years of age.

Why is Bronchiectasis Underdiagnosed?

The condition most often arises from several different causes, primarily an infection or disorder that leads to immunodeficiency in patients.  The most common symptom that is associated with a diagnosis is a persistent cough, which is a ubiquitous presentation in many respiratory ailments. This commonality is one of the primary reason it often goes undiagnosed, for currently healthcare providers in the developed world are not trained to make bronchiectasis part of their differential diagnosis work-up when assessing patients in clinical practice.

Clinical Results

Of the 236 patients enrolled in the study all with recurrent lower respiratory tract infections and non-CF bronchiectasis, the following characteristics were observed:

• 3% of the patients were female
• The average age was 65.7 years old
• 8% of the patients had never smoked
• 15% were current smokers
• FEV-1(forced expiratory volume in the first second) % of predicted was on average 87
• FVC (forced vital capacity) – % of predicted was on average 97
• The age of first clinical presentation was on average 58.3
• 4% of the patients were asthmatic
• 3% of the patients had allergic bronchopulmonary aspergillosis
• 7% of the patients had an auto-inflammatory disorder such as rheumatoid arthritis
• 1% of the patients had an immune deficiency disease such as HIV/AIDS

When discussing the importance of these findings the authors wrote, “In conclusion, the broad range of diseases that cause or coincide with bronchiectasis make it a frequently encountered entity in various medical specializations. The authors hope that this article will renew awareness of this still underdiagnosed condition. Exciting new developments are the publication of high-quality, randomized studies and new tools for patient selection which are important steps towards improving bronchiectasis management.”