Yoon Jung Oh,M.D. Departments of Pulmonary and Critical Care Medicine

1. Bronchiectasis Yoon Jung Oh,M.D. Departments of Pulmonary and Critical Care Medicine Ajou University School of Medicine

2. Definition Abnormal and permanent dilatation of medium sized( ≥ 2mm) bronchus by destruction of muscular, cartilage and elastic tissue component Three patterns of bronchiectasis (by Reid in 1950) ⑴ Cylindrical bronchiectasis ⑵ Cystic bronchiectasis ⑶ Varicose bronchiectasis Involved site ⑴ Lt.lower lobe post.basal segment (common) ⑵ Rt.middle lobe ⑶ bilateral (30%)

3. CaseM/30 상기 30세 남환은 수일 전 감기를 앓은 후 내원 당일 고열과 화농성 객담을 동반한 기침을 주소로 내원하였다. 환자는 부비동염으로 이비인후과에서 치료를 자주 받았다하며, 1년전에도 폐렴으로 입원한적이 있었다 한다. 평소 간헐적인 객혈이 있었으나 결핵의 기왕력은 없었다. P/E moist crackles on left lower lung field

4. Bronchiectasis

5. Cystic Bronchiectasis

6. Cystic Bronchiectasis Bronchogram HRCT

7. Bronchiectasis

8. Cylindrical Bronchiectasis

9. Varicose Bronchiectasis

10. Pathogenesis and Predisposing Factors • Bronchopulmonary infections • Hereditary abnormalities • Immunodeficiency states • Bronchial obstruction • Congenital anatomic defects • Miscellaneous disorders

11. 연령에 따른 원인 Childhood • Post pneumonia (Measle, Pertussis, Tbc) • Mechanical obstruction(foreign body) • Cystic fibrosis • -globulin deficiency(cong./aquired) • Immotile cilia syndrome Adulthood 1. Post pneumonia(viral, aspiration) 2. Pulmonary tuberculosis 3. Asthma, ABPA(allergic bronchopulmonary aspergillosis) 4. Toxic fume or congenital 5. Neoplasm, Chagas’ disease

12. Bronchopulmonary infections pertussis; measles S.aureus, Klebsiella, M.tuberculosis, H.influenaze adenovirus, influenza, H.simplex, HIV Mycotic infections Mycolplasmal infections Nontuberculous mycobacteria • Childhood diseases • Other bacterial infections • Other viral infections • Miscellaneous infections

13. Hereditary Abnormalities • Ciliary defects of respiratory mucosa • Kartagener’s syndrome • : bronchiectasis, situs inversus, sinusitis • Immotile cilia syndrome • : male infertility, defect in dynein arm or radical spoke • 1-Antitrypsin deficiency • : panacinar emphysema, bronchiectasis • Cystic fibrosis

14. Kartagener’s Syndrome • Triad • bronchiectasis, sinusitis, situs inversus • Incidence : 1/68,000 • Inheritance : Autosomal recessive trait • Pathogenesis • * absent or defective dysnein arms • * functional abnormality • (e.g., perhaps dysnein ATPase)

15. Kartagener’s syndrome

16. Immotile Cilia Sydrome Diagnostic Criteria 1. Recurrent & Chronic URI and LRI : rhinitis, sinusitis, otitis, bronchitis, bronchiectasis 2. Absence or near absence of tracheobronchial or nasal mucociliary transport 3. Total or near-total absence of dynein arms of the cilia in nasal or bronchial mucosa 4. Sterility in males

17. Normal Cilia MT : microtubules C : central sheath R : radial spokes D : dysnein arms N : nexin

18. Schematic cross section views of cilia A : Normal cilium Nine outer pairs of MT around a central pair B – D : congenital ciliary defects B : lacking dysnein arms C : missing radial spokes D : abnormal transposition

19. Tuberculosis and Bronchiectasis • Marked degree of caseation necrosis of bronchial walls, particularly when upper lobes are invaded • 2. Scarring of larger bronchi can produce bronchial stenosis • 3. Extraluminal obstruction of larger bronchi by tuberculous hilar lymphadenopathy • 4. Penetration by a calcified tuberculous node into the airway and broncholith formation • 5. Healed tuberculous cavities

20. Clinical Features of Bronchiectasis Signs crackles finger clubbing cyanosis cor pulmonale Symtoms sputum chronic cough hemoptysis dyspnea fever chest pain

21. Diagnosis • History / Physical examination • Plain chest X-ray : normal finding in 20% • HRCT(high resolution CT) • Bronchogram • Indications • ① Preoperative evaluation of unilateral or segmental disease previously identified on CT • ② Postoperativ evaluation of surgical airway complications such as dehiscence or fistula formation

22. Differential Diagnosis • Chronic bronchitis • Endobronchial tuberculosis • Endobronchial adenoma • Right middle lobe syndrome • Cystic fibrosis • Allergic bronchopulmonary aspergillosis • Pulmonary sequestration • Congenital abnormalities of the trachiobronchial tree

23. Aim of Treatment • Elimination of an identifiable underlying problem • Improved clearance of tracheobronchial secretions • Control of infection • Reversal of airflow obstruction

24. Treatment : Medical 1. Treatment of predisposing factors hypogammaglobulinemia  administration immunoglobulin tuberculosis  anti-tbc medication ABPA  steroid 2.Bronchial drainage : most important PDPV(postural drainage, percussion, vibration) PEEP deep brathing, continued coughing 3. Antibiotics

25. Treatment : Surgical Indication Contraindication Diffuse fibrosis or COPD Bilateral bronchiectasis Bronchiectasis complicating asthma Low FEV1/FVC Persisting predisposing factors (e.g. immunodeficiency) Localized bronchiectasis Massive/frequent hemoptysis Intractable infection Long standing collapse Adequate residual lung function

26. Complications • Recurrent pneumonia, sinusitis • Lung abscess • Pleuritis, empyema • Emphysema • Brain abscess(rare, but 10% of mortality) • Amyloidosis • Cor pulmonale(common in Korea) • Massive hemoptysis(rare)

27. Cystic Fibrosis • Incidence : Caucasian 1/1,500 – 2,000 • Asian 1/90,000 • Inheritance : Autosomal recessive trait • Pathogenesis • Single gene disorder • defective c-AMP mediated regulation of chloride channels • -> failure to secete chloride toward the lumen • -> unusually viscid mucous secretions • -> chronic pulmonary disease and pancreatic insufficiency

28. Cystic fibrosis

29. Diagnosis of cystic fibrosis 1. Sweat chloride test > 60 mEq : confirmative 50 – 60 mEq : highly probable < 50 mEq : normal 2. Chronic pulmonary disease (99%) 3. Pancreatic insufficiency(80-90%) 4. CXR increased interstitial marking (98%) cystic bronchiectasis (64%) hyperinflation(58%)

30. Treatment of cystic fibrosis Infection control pseudomonas(80%) S.aureus, H.influenza Bronchial drainage postural drainage, percussion and vibration forced exhalation technique positive expiratory pressure