Bronchiectasis

Definition:

 Abnormal permanent anatomical dilatation of bronchioles that cannot be cured or reversed. 

  • The large bronchi become inflamed, and walls become thickened, accompanied with

mucociliary dysfunction.

Epidemiology:

A rare disease nowadays, due to effective treatment of pulmonary infections.

  • The prevalence of bronchiectasis increases with age.

  • Onset of the process is usually at childhood. 

Causes:

Cystic fibrosis (CF):

  • The most common cause, accounting for more than 50% of cases. 

Other:
Recurrent infections: tuberculosis, pneumonia, and lung abscess.

  • Pan-hypogammaglobulnemia or immune deficiency.

  • Allergic bronchopulmonary asperigellosis (ABPA).

  • Collagen-vascular disease (rheumatoid arthritis RA, or Sjögren syndrome).

  • Lung tumors or foreign body.

  • Primary ciliary dyskinesia (PCD).

  • Idiopathic. 

 

 

Types:

Study guide:

Bronchiectasis

Diffused

Associated with:

1- CF

2- Immune deficiency

3- PCD

4-  RA or Sjogren syndrome

5- ABPA

6- Idiopathic

Focal

Associated with:

1- Untreated pneumonia

2- Obstruction due to tumor or foreign body 

3- Mycobacteria

Pathophysiology:

Recurrent infections will trigger the neutrophils & cause inflammation.

  • Inflammatory mediators destroy elastin, muscles, and cartilage in the large & medium airwaysè irreversible bronchodilation. 

  • Lymphocytes and macrophages will infiltrate the mucosal walls and cause thickening, which is responsible for the airway obstruction.

  • With time, the disease will progress and spread to the lung parenchyma causing fibrosis.

  • Impaired airway clearance mechanism will also contribute to the airway obstruction.

  • Various organisms will frequently colonize chronically dilated bronchi.

Clinical Presentation: (can be difficult to differentiate from chronic bronchitis)

Recurrent mucopurulent (khaki colored) foul smelling productive cough, in large amounts.

  • Hemoptysis: due to rupture of the bronchial arteries.

  • Dyspnea and wheezes.

  • Weight loss.

  • Coarse crackles.

  • Digital clubbing (rare).

  • Signs of anemia (anemia of chronic diseases).

Diagnosis:

Chest X-ray (CXR): 

  • The best initial test.

  • Dilated thickened bronchi (tram-track appearance).

    • High-resolution CT: gold stabdard

      • The most accurate diagnostic test (nearly 100% sensitive & specific).

      • Airways are larger than their associated vessels.

      • Dilated thickened bronchi (signet ring appearance).

    •  Sputum culture & gram staining:

      • Important for effective treatment.

      • Major pathogens are:

        • H.influenzae (35%) 

        • P.aeruginosa (31%)

        • M.catarrhalis (20%) 

        • S.aureus (14%, especially in CF patients)

        • Anaerobes

        • Others: S.pneumoniae, K.pneumoniae, aspergillus fumigatus, M.avium. 

    • Sweat chloride test: 

      • If cystic fibrosis is suspected. 

treatment:

Chest physiotherapy and postural drainage:

  • Cupping and clapping.

  • At least 3 times daily for 10-20 minutes.

    • Antibiotics:

      • Oral, IV, or inhaled.

      • Rotate antibiotics, 1 drug weekly each month.

      • Choices:

        • Macrolides (azithromycin, or clarithromycin).

        • Cephalosporins (Cefuroxime, cefaclor, or cefixime).

        • Quinolones (levofloxacin, or moxifloxacin).

    • Bronchodilators: in patients with airflow limitation.

    • Mucolytics

    • Oxygen

    • Inhaled or oral steroids: to delay disease progression and for ABPA.

    • Surgical resection of localized lesions (rarely used). 

 

Complications:

  • Pneumonia.

  • Empyema.

  • Pneumothorax.

  • Metastatic cerebral abscess.

  • Massive hemoptysis:

    • Mortality is 25%.

    • Due to rupture of high-pressure systemic bronchial arteries.

    • Usually self-limited, if did not stop bronchial artery embolization is the treatment of choice. 

 

Prognosis:

Patients with cystic fibrosis have the poorest outcome.

  • Most bronchiectasis patients will eventually develop respiratory failure or cor pulmonale. 

References:

  1. Kumar P, Clark M. Kumar & Clark's clinical medicine.

  2. Walker B, Colledge N, Ralston S, Penman I. Davidson's principles and practice of medicine.

  3. Le T, Bhushan V, Singh Bagga H. First aid for the USMLE step 2 CK. New York: McGraw-Hill Medical; 2010.

  4. Fischer C. Master the boards.

  5. Agabegi S, Agabegi E, Ring A. Step-up to medicine. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins; 2013.

  6. Bilton D, Jones A. [Internet]. 2016 [cited 23 January 2016]. Available from: http://www.sppneumologia.pt/uploads/files/gruposdeestudo/Núcleo%20de%20Estudos%20de%20Bronquiectasias%20Não%20Fibrose%20Qu%C3%ADstica/PDF75

  7. 276. Merck Manuals Professional Edition. Bronchiectasis - Pulmonary Disorders [Internet]. 2016 [cited 23 January 2016]. Available from: http://www.merckmanuals.com/professional/pulmonary-disorders/bronchiectasis-and-atelectasis/bronchiectasis

  8. Flickr - Photo Sharing. Sarcoidosis - Bronchiectasis [Internet]. 2016 [cited 23 January 2016]. Available from: https://www.flickr.com/photos/pulmonary_pathology/6076312297 (Figure 1). 

  9. Gaillard F. Bronchiectasis | Radiology Reference Article | Radiopaedia.org [Internet]. Radiopaedia.org. 2016 [cited 23 January 2016]. Available from: http://radiopaedia.org/articles/bronchiectasis (Figure 2).

  10. Quizlet.com. COPD and Bronchiectasis flashcards | Quizlet [Internet]. 2016 [cited 23 January 2016]. Available from: https://quizlet.com/22288474/copd-and-bronchiectasis-flash-cards/ (Figure 3). 

  11. Mobasser SS. DEJA REVIEW Internal Medicine, 2nd ed.; 2011.

Written by:         Roaa Amer    
​                                                                    
Reviewed by:     Haifa Al Issa
                                   Areej Madani

​Format editor:  Salman Alahmed

​Audio Recording:


- Read by: Bayan Alzomili


- Directed by: Rana Alzahrani


- Audio production: Bayan Alzomaili