FlashPath - Lung - Bronchiectasis
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This document discusses bronchiectasis, an obstructive lung disease characterized by permanent dilation of the airways. It causes chronic cough with copious sputum production. Common causes include cystic fibrosis, infections from bacteria/viruses/fungi, and primary ciliary dyskinesia. High-resolution CT is the best way to diagnose bronchiectasis and classify its appearance. Pathology shows dilated airways filled with mucus, inflammation, necrosis, fibrosis and squamous metaplasia of the airway lining. Differential diagnosis includes other obstructive lung diseases and congenital lung abnormalities.
FlashPath - Lung - Bronchiectasis
- 1. FLASHPATH H a z e m A l i
- 2. BRONCHIECTASIS H a z e m A l i
- 3. CLINICAL Bronchiectasis is one of the “obstructive lung diseases” that include: • Chronic bronchitis • Emphysema • Small-airway disease “bronchiolitis” • Asthma
- 4. CLINICAL Obstructive airway disease Restrictive airway disease General features Increase in resistance to airflow due to obstruction at any level Reduced expansion of lung parenchyma Total lung capacity (TLC) Increased Reduced Forced Expiratory Volume in one second (FEV1) Reduced Normal
- 5. CLINICAL • Bronchiectasis is permanent dilation and destruction of the airways – 2ry to recurrent infections and bronchial obstruction – Mainly affect bronchi and bronchioles proximal to the terminal bronchioles bronchioles • Usually present with: – Chronic, productive cough • Copious amount of foul smelling, purulent sputum – Dyspnea – Fever – Others: of the original cause / associated complications
- 6. CLINICAL • High-resolution CT is the procedure of choice for noninvasive diagnosis – Bronchiectasis is radiologically classified into: • Cylindrical • Saccular • Cystic • Effective antibiotic therapy has led to a marked decrease in the incidence of bronchiectasis and its complications: – Bronchopleural fistula with empyema – Brain abscess – 2ry (systemic) Amyloidosis
- 7. CLINICAL Causes of bronchiectasis Congenital Necrotizing Pneumonia Bronchial Obstruction Inflammatory Disorders Idiopathic • Cystic fibrosis • Intralobar sequestration • Primary ciliary dyskinesia • Kartagener syndromes • Bacteria • Staph, Strept • Pseudomonas • TB • Viruses • Pertussis • Measles • Fungi • True obstruction: • Tumor • Foreign bodies • Mucus impaction • Functional obstruction: • COPD • RA • SLE • IBD • Transplant rejection
- 8. CLINICAL • Cystic fibrosis is commonly associated with Bronchiectasis, due to: – Obstruction (mucus plugs) – Infection (decreased ciliary clearance of bacteria) • Defective ciliary function (dyskinesia) is also associated with Bronchiectasis, e.g. Kartegener syndrome: – Autosomal recessive – Absent or irregular dynein arms (motor part) of cilia – Defective bacterial clearance (bronchiectasis, sinusitis) – Defective cell motility during embryogenesis (situs inversus) – Defective sperm movement (male infertility)
- 9. CLINICAL • Aspergilloma (fungal ball) can occur within pulmonary cavities (e.g. prior TB, Bronchiectasis, old Infarcts, or lung Abscesses). • Allergic bronchopulmonary aspergillosis (occurs with asthma and cystic fibrosis) may lead to proximal bronchiectasis and fibrotic lung disease
- 10. PATHOGENESIS Accumulation of Secretions Secondary Infection Inflammation, Necrosis, Fibrosis Permanent Dilatation of bronchi More Accumulation of Secretions Bronchial Obstruction
- 11. GROSS • Mainly noticed at the lower lobes • Bilateral in 50% of cases • Bronchi are markedly dilated – Diameter of the bronchus exceeds the diameter of the accompanying bronchial artery – Can form cystic spaces • Bronchi are filled with yellowish-greenish secretions
- 12. GROSS • Sometimes the dilated bronchi can extend out to the pleural surface – Normally, the bronchioles cannot be followed by eye beyond a point 2 to 3 cm from the pleural surfaces Also other features of Cause/Complications: • Pneumonia • Lung abscesses • Empyema • Aspergillosis • Tumor • Foreign body
- 13. MICROSCOPY Airways: • Bronchial Architecture: – Dilated (may be cystic) – Mucopurulent exudate • Mucosa: – Erosion/Ulceration – Squamous metaplasia • Wall: – Chronic Inflammation (+/- prominent lymphoid follicles) – Destruction of cartilage / muscle / submucosal glands – Fibrosis Also other features of Cause/Complications: • Pneumonia • Lung abscesses • Empyema • Aspergillosis • Tumor • Foreign body
- 14. CYTOLOGY In long-standing bronchiectasis you get atypical squamous metaplasia and isolated bizarre squamous cells – You need numerous bizarre cells to diagnose SCC with confidence
- 15. DIFFERENTIAL DIAGNOSIS Chronic bronchitis Bronchiectasis Asthma Small-airway disease “bronchiolitis” Emphysema Site L a r g e a i r w a y s ( B r o n c h i ) Bronchioles Alveoli Major pathology • Mucous gland hyperplasia • Excess mucus • Inflammation • Airway dilation & scarring • Thickened basement membrane • Smooth muscle hyperplasia • Excess mucus • Inflammation (eosinophils) • Inflammatory scarring & obliteration • Airspace enlargement • Wall destruction • No fibrosis Other obstructive lung diseases:
- 16. DIFFERENTIAL DIAGNOSIS Other causes of chronic cough • Lung carcinoma • Bronchiectasis • Cystic fibrosis • Congestive heart failure • Tuberculosis
- 17. DIFFERENTIAL DIAGNOSIS O t h e r c o n g e n i t a l / c y s t i c l u n g d i s e a s e s : • Congenital: – Bronchogenic cysts – Congenital pulmonary cysts – Congenital pulmonary airway malformation – Congenital lobar emphysema – Pulmonary sequestration • Acquired: – Healed abscess – Honeycombing – Emphysema
- 18. DIFFERENTIAL DIAGNOSIS Middle lobe syndrome: • Chronic or recurrent atelectasis (radiographic opacification) of the right middle lobe, lingula, or both • Mainly due to bronchial obstruction: – Enlarged lymph nodes – Tumor • Combinations of the following features: – Atelectasis – Bronchiectasis – Chronic inflammation (+/- Lymphoid hyperplasia) – Organizing pneumonia – Abscesses – Granulomatous inflammation
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- 20. THANK YOU H a z e m A l i