Diagnosing non-CF bronchiectasis
Due to the commonality of symptoms and relatively rare prevalence, non-CF bronchiectasis diagnosis can be a long journey with several stages. To avoid costly delays, non-CF bronchiectasis should be considered in all patients with excessive sputum production, chronic cough, or rapid/frequent relapse of respiratory infections following a course of antibiotics.1-3
Chest x-rays can show signs of non-CF bronchiectasis, but the diagnosis can only be confirmed by high-resolution CT scan.4,5
Donna struggled with daily symptoms and respiratory infections for years before being diagnosed with non-CF bronchiectasis.
GO BEYOND THE PATIENT RECORD
WHEN DIAGNOSING NON-CF BRONCHIECTASIS
Donna, 59-year-old nonsmoker
- Part-time librarian
- Social and active, but increasingly limited by her condition
PATIENT HISTORY
- 2+ years of recurring symptoms and respiratory infections
- Intermittent dyspnea
- Diagnosed with exercise-induced asthma
- Prescribed short-acting beta-agonist
- Increasingly chronic cough with purulent sputum
- Changed PCP 8 months ago due to unresolved symptoms
"I was so tired of my symptoms coming back again and again. I ended up changing from a doctor I had been seeing for nearly 10 years to try to find answers."
KEY CLINICAL INSIGHT
Sputum expectoration averages 1–3 oz/day, and can reach maximum volumes of 6–19 oz/day.6
GO BEYOND THE PATIENT RECORD
WHEN DIAGNOSING NON-CF BRONCHIECTASIS
RECENT PHYSICIAN VISITS
- Multiple visits for 2 lung infections over 5-month span
- Initial bronchial infection required 2 courses of antibiotic therapy
- Subsequent infection required ER visit while on vacation
- Debilitating dyspnea and fatigue
- Concerning hemoptysis
- Infection marked by significant sputum expectoration
- Advised to visit a pulmonologist after returning home
"I was worried about coughing and breathing being an issue on my trip, only to end up in an ER in a city far from home."
KEY CLINICAL INSIGHT
Persistent or recurring lung infections, may be an indication of non-CF bronchiectasis.3
GO BEYOND THE PATIENT RECORD
WHEN DIAGNOSING NON-CF BRONCHIECTASIS
PULMONOLOGIST REFERRAL
- Preliminary workup suggestive of bronchiectasis
- Chest x-ray showed ill-defined nodular opacities
- Culture positive for P aeruginosa
- Diagnosis of non-CF bronchiectasis confirmed by HRCT
- Lower left lobe showed bronchiectatic changes
- Dilated and fluid-filled bronchi
"There was some comfort in finally having a correct diagnosis, though my doctor said there was no FDA-approved medicine for it. I was hopeful this diagnosis would be the first step to fewer exacerbations."
KEY CLINICAL INSIGHT
As isolation of P aeruginosa is NOT common in the sputum of COPD patients, non-CF bronchiectasis should be considered when present.6
Sputum culture testing is an important step that can guide an appropriate treatment plan.5
GO BEYOND THE PATIENT RECORD
WHEN DIAGNOSING NON-CF BRONCHIECTASIS
ACUTE RECOMMENDATION
- Attempt to reduce/eliminate presence of underlying pathogen
- Oral ciprofloxacin 750 mg twice daily for 2 weeks
ONGOING RECOMMENDATION
- Azithromycin 500 mg 3 times/week
- Short-acting beta-agonist as needed
- Airway clearance exercises
- Hyperosmolar agent
- Chest physiotherapy
"I want to do whatever I can to limit the impact of non-CF bronchiectasis, reduce my exacerbations, and stay out of the ER."
While challenges remain in non-CF bronchiectasis, patients can benefit from early diagnosis, awareness of underlying pathogens, and airway clearance techniques.
Managing non-CF bronchiectasis
You can successfully manage bronchiectasis if you lead and live a healthy, active lifestyle.
Patients feel frustrated, confused, and helpless—often waiting months or even years for a diagnosis.
While there are currently no approved pharmacologic therapies for chronic non-CF bronchiectasis treatment, there are guidelines for management. Sources include the British Thoracic Society (BTS), Spanish Society of Pulmonology and Thoracic Surgery (SEPAR), European Respiratory Society (ERS), and European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC). There are currently no official non-CF bronchiectasis guidelines in the United States.
The British Thoracic Society divides the management, principles, and general approach to non-CF bronchiectasis into 7 categories.6
The general approach in non-CF bronchiectasis is to identify and treat the underlying cause to prevent disease progression, with the goal of controlling symptoms, enhancing quality of life, reducing exacerbations, and maintaining pulmonary function.6
Because early treatment of exacerbations is recommended, it is important to ensure that patients understand the basic principles of disease management. It can be helpful to give patients a written explanation of the disease, the role of infection, and how to recognize exacerbations.6
Techniques for helping patients expectorate, or release sputum, can help alleviate daily symptoms. Respiratory physiotherapy, including ACTs, plays a critical role in management of non-CF bronchiectasis.6
Most agents used to treat non-CF bronchiectasis attack the physical properties of the mucus to aid in airway clearance, and are sometimes used in conjunction with antibiotics and physiotherapy.6
Antibiotics are recommended for exacerbations that present over several days with worsening symptoms. The choice of antibiotic agent should be determined by identification of the pathogenic bacteria through a sputum culture.6
When conservative intervention and management techniques fail, leaving patients facing recurrent respiratory infections, excessive sputum production, chronic cough, and persistent lung abscess, surgical intervention may be considered.6
Some patients with advanced disease may benefit from therapy to manage disease complications. While few long-term studies have been conducted, treatments such as noninvasive ventilation (NIV) and oxygen therapy may improve quality of life.6
Physiotherapy and lifestyle changes
Respiratory physiotherapy, along with exercise and nutritional advice, may play an important role in the daily quality of life for patients with non-CF bronchiectasis. Airway clearance techniques (ACTs) aid in the mobilization and expectoration of bronchopulmonary secretions, and can improve efficiency of ventilation and exercise tolerance, and reduce breathlessness and thoracic pain.6
There are many ACTs used for non-CF bronchiectasis management. Although techniques vary, traditional ACTs have four common components7:
- postural drainage
- percussion
- vibration of the chest wall
- proper coughing techniques
Certain types of exercise improve cardiac conditioning and mobilization of airway secretions in non-CF bronchiectasis. Patients who participate in regular exercise programs demonstrate significant improvement in exertional tolerance and health-related quality of life scores, and experience fewer exacerbations.8
Poor nutrition may be associated with impaired immune function. And while it is not typically listed as an etiologic factor in bronchiectasis, malnutrition may contribute to its occurrence. The Bronchiectasis Severity Index (BSI) considers body mass index when classifying patients.9-11
References
- Chawla K, Vishwanath S, Manu M, Lazer B. Influence of Pseudomonas aeruginosa on exacerbation in patients with bronchiectasis. J Global Infect Dis. 2015;7:18-22.
- Yap VL, Metersky ML. New therapeutic options for noncystic fibrosis bronchiectasis. Curr Opin Infect Dis. 2015;28(2):171-176.
- Altenburg J, Wortel K, Van der Werf TS, Boersma WG. Non-cystic fibrosis bronchiectasis: clinical presentation, diagnosis and treatment, illustrated by data from a Dutch teaching hospital. Neth J Med. 2015;73:147-154.
- Athanazio R. Airway disease: similarities and differences between asthma, COPD and bronchiectasis. Clinics. 2012;67(11):1335-1343.
- McShane PJ, Naureckas ET, Tino G, Strek M. Non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med. 2013;188:647-656.
- British Thoracic Society Bronchiectasis (non-CF) Guideline Group. Guideline for non-CF bronchiectasis. Thorax. 2010;65:suppl.
- Snijders D, Dominguez BF, Calgaro S, et al. Mucociliary clearance techniques for treating non-cystic fibrosis bronchiectasis: is there evidence? Int J Immunopathol Pharmacol. 2015;28:150-159.
- ElMaraachli W, Conrad DJ, Wang A. Using cystic fibrosis therapies for non–cystic fibrosis bronchiectasis. Clin Chest Med. 2016;37:139-146.
- King PT. The pathophysiology of bronchiectasis. Int J Chron Obstruct Dis. 2009;4:411-419.
- Jin J, Yu W, Li S, Lu L, Liu X, Sun Y. Factors associated with bronchiectasis in patients with moderate severe chronic obstructive pulmonary disease. Medicine. 2016;95:e4219.
- McDonnell MJ, Aliberti S, Goeminne PC, et al. Multidimensional severity assessment in bronchiectasis: an analysis of seven European cohorts. Thorax. 2016;71:1110-1118.