Lecture
Patient will present as → a 25-year-old cystic fibrosis patient complaining of chronic, frequent coughing productive of yellow and green sputum. She recently recovered from a Pseudomonas spp. Pneumonia requiring hospitalization. On physical examination, you notice foul breath, purulent sputum, and hemoptysis, along with a CXR demonstrating dilated and thickened airways with “tram-track” lines and ring shadows.
To watch this and all of Joe-Gilboy PA-C's video lessons you must be a member. Members can log in here or join now.
Bronchiectasis is permanent dilation and destruction of the bronchial walls - the common endpoint of many disorders that cause chronic airway inflammation
- A "vicious cycle": infection + impaired mucus clearance → inflammation → airway wall destruction → more mucus, bacterial colonization, and infection
Causes
- In adults, most cases are post-infectious (prior pneumonia, pertussis, tuberculosis, nontuberculous mycobacteria) or idiopathic; childhood viral infections (adenovirus, influenza, measles) are classic triggers
- Other causes: COPD, immunodeficiency (e.g., common variable immunodeficiency), allergic bronchopulmonary aspergillosis (ABPA), rheumatoid arthritis, primary ciliary dyskinesia (Kartagener syndrome - situs inversus, sinusitis, infertility), alpha-1 antitrypsin deficiency, and focal obstruction such as foreign body aspiration or tumor
- Cystic fibrosis is a classic cause (upper-lobe disease) - consider it in younger patients and test for it
Who gets it and how it presents
- More common in women and rises steeply with age
- Daily cough with copious, purulent (often foul-smelling) sputum for months to years, with recurrent chest infections, dyspnea, and fatigue
- Hemoptysis is common and usually mild (from bronchial arteries); massive hemoptysis is rare but can be life-threatening
- Crackles are common on exam; clubbing is uncommon
Organisms
- Haemophilus influenzae and Pseudomonas aeruginosa are the most commonly isolated bacteria
- Chronic P. aeruginosa infection signals more severe disease and predicts more exacerbations, admissions, and death
CXR (first test) – linear ("tram track") lung markings and ring shadows from dilated, thickened airways (often nonspecific, and may be normal)
- The diagnostic test of choice is a noncontrast high-resolution CT of the chest - airway wider than its adjacent artery (signet ring sign), thickened airway walls, lack of tapering, and airways visible near the pleura
- Distribution clues: upper lobes = cystic fibrosis; central (perihilar) = ABPA; middle lobe and lingula = nontuberculous mycobacteria
- Look for the cause: CBC, immunoglobulin levels (IgG, IgA, IgM), sputum culture (including mycobacteria), sweat chloride if cystic fibrosis is possible, and Aspergillus IgE if ABPA is possible
- Pulmonary function tests usually show an obstructive pattern
Chest X-ray of hyperinflated lung with bronchiectasis at the right upper lobe of a 12-year-old boy.
A CT scan demonstrating very pronounced bronchiectasis in a 75-year-old man, especially on the right (left in the picture). There is a florid superinfection with a large accompanying effusion
Airway clearance is the cornerstone of daily treatment
- Chest physiotherapy and airway clearance devices daily (± hypertonic saline), pulmonary rehabilitation, and vaccines (influenza, pneumococcal)
- Exacerbations → send sputum culture, then 14 days of antibiotics chosen by prior sputum cultures
- H. influenzae → amoxicillin, amoxicillin-clavulanate, or doxycycline; Pseudomonas → ciprofloxacin (IV antipseudomonal antibiotic if severe or resistant)
- 3 or more exacerbations per year → long-term macrolide (azithromycin) after ruling out nontuberculous mycobacteria; inhaled antibiotics for chronic Pseudomonas; brensocatib (oral DPP-1 inhibitor) is a newer add-on that reduces exacerbations
- Treat the cause: immunoglobulin replacement for immunodeficiency, ABPA treatment, alpha-1 antitrypsin replacement
- Avoid recombinant DNase (dornase alfa) in non-cystic fibrosis bronchiectasis (it can be harmful) and routine inhaled corticosteroids (unless asthma or COPD)
- Surgery for localized disease; lung transplant for advanced, refractory disease
Flashcards ready when you areYou have opened several Quizlet sets recently. Loading this one only when needed helps prevent a temporary Quizlet limit.
 Osmosis |
|
 |
Picmonic
Bronchiectasis
Bronchiectasis is permanent airway dilation caused by chronic infection and inflammation, sometimes from bronchial obstruction. The subsequent muscle and elastic tissue damage permanently dilates the airways, which may trap air in the lungs and lead to collapse of the bronchioles. Common symptoms include increased purulent sputum production and hemoptysis. In adults, most cases follow prior lung infections or are idiopathic. Poor ciliary motility from congenital causes like Kartagener’s Syndrome (primary ciliary dyskinesia) is a classic association. Other predisposing conditions are cystic fibrosis, which impairs clearance of mucus from the airways, and allergic bronchopulmonary aspergillosis (ABPA), an immune response to Aspergillus fungus.
Play Video + QuizQuestion 1 |
A 61-year-old woman has had a cough productive of thick sputum most days for 3 years, with several chest infections. Noncontrast high-resolution CT of the chest shows dilated, thick-walled bronchi in both lower lobes. In adults with bronchiectasis in whom a cause is found, which of the following is the most common cause?
Allergic bronchopulmonary aspergillosis Hint: ABPA causes central (perihilar) bronchiectasis in patients with asthma or cystic fibrosis, but it is an uncommon cause overall. | |
Alpha-1 antitrypsin deficiency Hint: Alpha-1 antitrypsin deficiency is a recognized but uncommon cause; it is checked in selected patients. | |
Cystic fibrosis Hint: Cystic fibrosis is a classic cause (upper-lobe disease, often diagnosed in childhood or young adulthood), but it accounts for a minority of adult cases. | |
Postinfectious airway injury | |
Primary ciliary dyskinesia Hint: Primary ciliary dyskinesia (Kartagener syndrome) is a rare cause that usually presents in childhood with sinus and ear disease. |
Question 1 Explanation:
Postinfectious airway injury (prior severe pneumonia, pertussis, measles, tuberculosis, or nontuberculous mycobacteria) is the most common identified cause of bronchiectasis in adults, and many cases remain idiopathic after workup. Bronchiectasis is the common end point of a "vicious cycle": an infectious insult plus impaired mucus clearance leads to inflammation, airway wall destruction, permanent dilation, and more infection. Other causes to look for include COPD, immunodeficiency (e.g., common variable immunodeficiency), ABPA, rheumatoid arthritis, primary ciliary dyskinesia, alpha-1 antitrypsin deficiency, and cystic fibrosis. Teaching cystic fibrosis as "the most common cause" in adults is outdated.
Question 2 |
A 69-year-old man with chronic lymphocytic leukemia has a 2-year history of daily cough with copious purulent sputum and recurrent chest infections. Crackles are heard at both lung bases. Chest x-ray shows parallel "tram-track" lines and ring shadows in the lower lobes. Which of the following is the most appropriate next step to confirm the diagnosis?
Bronchoscopy Hint: Bronchoscopy can help with cultures or hemoptysis but does not show the airway dilation that defines the diagnosis. | |
Noncontrast high-resolution CT of the chest | |
Pulmonary function testing Hint: Pulmonary function tests (usually an obstructive pattern) assess severity but cannot confirm bronchiectasis. | |
Sputum culture Hint: Sputum culture guides antibiotic choice but does not confirm the structural diagnosis. | |
Ventilation-perfusion scan Hint: A V/Q scan evaluates pulmonary embolism, not airway dilation. |
Question 2 Explanation:
Chronic daily productive cough with recurrent infections suggests bronchiectasis; hypogammaglobulinemia from CLL is a risk factor. Chest x-ray findings (tram tracks, ring shadows) are suggestive but insensitive. Noncontrast high-resolution (thin-section) CT is the diagnostic test of choice: an airway wider than its adjacent artery (signet ring sign), lack of bronchial tapering, and airways visible near the pleura. Once confirmed, look for the cause (CBC, immunoglobulin levels, sputum culture including mycobacteria, and selected tests such as sweat chloride or Aspergillus IgE).
Question 3 |
A 54-year-old woman with bronchiectasis has 4 days of increased sputum volume and purulence and worsening dyspnea. She is afebrile, and SpO2 is 95% on room air. Chest x-ray shows no new infiltrate. Her sputum cultures over the past year have repeatedly grown Pseudomonas aeruginosa sensitive to fluoroquinolones. A new sputum culture is sent. Which of the following is the most appropriate treatment?
Amoxicillin for 5 days Hint: Amoxicillin has no activity against Pseudomonas, and 5 days is too short for a bronchiectasis exacerbation. | |
Azithromycin for 5 days Hint: Azithromycin is used long term to prevent exacerbations; it does not treat a Pseudomonas exacerbation. | |
Ciprofloxacin for 14 days | |
Doxycycline for 7 days Hint: Doxycycline does not cover Pseudomonas. | |
Inhaled tobramycin for 14 days Hint: Inhaled antibiotics are used for long-term suppression of chronic Pseudomonas, not as sole treatment of an acute exacerbation. |
Question 3 Explanation:
Treat a bronchiectasis exacerbation with 14 days of antibiotics chosen by the patient's prior sputum cultures (send a new culture before starting). For Pseudomonas aeruginosa, oral ciprofloxacin is the usual first choice; use an IV antipseudomonal regimen if the illness is severe or the organism is resistant. Intensify airway clearance at the same time. Systemic steroids are not routinely used unless the patient also has asthma or COPD with wheeze.
Question 4 |
A 66-year-old man with idiopathic bronchiectasis has had 4 exacerbations requiring antibiotics in the past year despite daily airway clearance and up-to-date vaccines. Sputum cultures grow Haemophilus influenzae, and mycobacterial cultures are negative. His ECG shows a normal QT interval. Which of the following is the most appropriate long-term therapy to reduce exacerbations?
Inhaled budesonide Hint: Inhaled corticosteroids are not routinely used in bronchiectasis unless asthma or COPD is also present. | |
Long-term azithromycin | |
Oral prednisone Hint: Long-term systemic steroids add harm without reducing bronchiectasis exacerbations. | |
Recombinant human DNase (dornase alfa) Hint: Recombinant DNase helps in cystic fibrosis but is ineffective and potentially harmful in non-cystic fibrosis bronchiectasis. | |
Theophylline Hint: Theophylline has no role in preventing bronchiectasis exacerbations. |
Question 4 Explanation:
For adults with 3 or more exacerbations per year despite airway clearance, long-term macrolide therapy (azithromycin) reduces exacerbations. Before starting, exclude nontuberculous mycobacteria (macrolide monotherapy breeds resistance) and check for QT prolongation and hearing loss. For chronic Pseudomonas infection, a long-term inhaled antibiotic is an alternative. Brensocatib, an oral DPP-1 inhibitor, is a newer add-on that also reduces exacerbations.
Question 5 |
A 24-year-old man has had a daily wet cough since early childhood, chronic sinusitis, and recurrent ear infections. He and his partner have been unable to conceive. His heart sounds are loudest in the right side of the chest. CT of the chest shows bronchiectasis in the middle and lower lobes. Which of the following is the most likely diagnosis?
Allergic bronchopulmonary aspergillosis Hint: ABPA occurs in patients with asthma or cystic fibrosis and causes central bronchiectasis; it does not cause situs inversus. | |
Alpha-1 antitrypsin deficiency Hint: Alpha-1 antitrypsin deficiency causes lower-lobe emphysema and liver disease, not dextrocardia. | |
Common variable immunodeficiency Hint: Common variable immunodeficiency causes recurrent sinopulmonary infections but not situs inversus or immotile sperm. | |
Cystic fibrosis Hint: Cystic fibrosis causes bronchiectasis (upper-lobe predominant), sinusitis, and male infertility, but not situs inversus. | |
Primary ciliary dyskinesia |
Question 5 Explanation:
Primary ciliary dyskinesia (Kartagener syndrome when situs inversus is present) causes defective ciliary motion: lifelong wet cough, chronic sinusitis, otitis media, bronchiectasis (middle and lower lobes), and infertility from immotile sperm. Situs inversus (dextrocardia) occurs in about half of patients. Low nasal nitric oxide and ciliary testing support the diagnosis.
There are 5 questions to complete.
|
List |
References: Merck Manual · UpToDate