Bronchiectasis
Definition:
Abnormal permanent anatomical dilatation of bronchioles that cannot be cured or reversed.
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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.
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The prevalence of bronchiectasis increases with age.
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Onset of the process is usually at childhood.
Causes:
Cystic fibrosis (CF):
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The most common cause, accounting for more than 50% of cases.
Other:
Recurrent infections: tuberculosis, pneumonia, and lung abscess.
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Pan-hypogammaglobulnemia or immune deficiency.
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Allergic bronchopulmonary asperigellosis (ABPA).
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Collagen-vascular disease (rheumatoid arthritis RA, or Sjögren syndrome).
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Lung tumors or foreign body.
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Primary ciliary dyskinesia (PCD).
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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.
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Inflammatory mediators destroy elastin, muscles, and cartilage in the large & medium airwaysè irreversible bronchodilation.
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Lymphocytes and macrophages will infiltrate the mucosal walls and cause thickening, which is responsible for the airway obstruction.
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With time, the disease will progress and spread to the lung parenchyma causing fibrosis.
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Impaired airway clearance mechanism will also contribute to the airway obstruction.
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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.
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Hemoptysis: due to rupture of the bronchial arteries.
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Dyspnea and wheezes.
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Weight loss.
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Coarse crackles.
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Digital clubbing (rare).
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Signs of anemia (anemia of chronic diseases).
Diagnosis:
Chest X-ray (CXR):
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The best initial test.
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Dilated thickened bronchi (tram-track appearance).
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High-resolution CT: gold stabdard
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The most accurate diagnostic test (nearly 100% sensitive & specific).
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Airways are larger than their associated vessels.
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Dilated thickened bronchi (signet ring appearance).
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Sputum culture & gram staining:
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Important for effective treatment.
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Major pathogens are:
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H.influenzae (35%)
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P.aeruginosa (31%)
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M.catarrhalis (20%)
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S.aureus (14%, especially in CF patients)
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Anaerobes
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Others: S.pneumoniae, K.pneumoniae, aspergillus fumigatus, M.avium.
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Sweat chloride test:
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If cystic fibrosis is suspected.
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treatment:
Chest physiotherapy and postural drainage:
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Cupping and clapping.
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At least 3 times daily for 10-20 minutes.
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Antibiotics:
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Oral, IV, or inhaled.
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Rotate antibiotics, 1 drug weekly each month.
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Choices:
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Macrolides (azithromycin, or clarithromycin).
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Cephalosporins (Cefuroxime, cefaclor, or cefixime).
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Quinolones (levofloxacin, or moxifloxacin).
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Bronchodilators: in patients with airflow limitation.
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Mucolytics
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Oxygen
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Inhaled or oral steroids: to delay disease progression and for ABPA.
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Surgical resection of localized lesions (rarely used).
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Complications:
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Pneumonia.
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Empyema.
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Pneumothorax.
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Metastatic cerebral abscess.
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Massive hemoptysis:
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Mortality is 25%.
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Due to rupture of high-pressure systemic bronchial arteries.
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Usually self-limited, if did not stop bronchial artery embolization is the treatment of choice.
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Prognosis:
Patients with cystic fibrosis have the poorest outcome.
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Most bronchiectasis patients will eventually develop respiratory failure or cor pulmonale.
References:
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Kumar P, Clark M. Kumar & Clark's clinical medicine.
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Walker B, Colledge N, Ralston S, Penman I. Davidson's principles and practice of medicine.
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Le T, Bhushan V, Singh Bagga H. First aid for the USMLE step 2 CK. New York: McGraw-Hill Medical; 2010.
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Fischer C. Master the boards.
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Agabegi S, Agabegi E, Ring A. Step-up to medicine. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins; 2013.
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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
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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
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Flickr - Photo Sharing. Sarcoidosis - Bronchiectasis [Internet]. 2016 [cited 23 January 2016]. Available from: https://www.flickr.com/photos/pulmonary_pathology/6076312297 (Figure 1).
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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).
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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).
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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