1. Clinical manifestation and diagnosis of bronchiectasis Aleš Rozman University Clinic of Respiratory Diseases and Allergy, GOLNIK, Slovenia Portorož – 9th May 2009

2. Bronchiectasis: - refers to a permanent abnormal dilatation of the bronchi and bronchioli, caused by recurrent infections which destruct muscular and elastic components of bronchial walls.

3. 1. Epidemiology approximately 40 /100.000 (est.) more in women more in elderly population more in societies with pure access to health care

4. 2. Etiologies infection of the airway + susceptibility Susceptibility: 1.airway obstruction 2.defect in host defence 3.impaired drainage 4.other

5. 2. Etiologies – airway obstruction Innate: bronchomalacia tracheobronchomegaly bronchial cyst ectopic bronch pulmonary sequestration Yellow nail sy. Acquired foreign body aspiration (children,...) (benign) tumour hilar adenopathy (TBC, sarcoidosis) chronic bronchitis polychondritis mucus impaction (ABPA,...)

6. 2. Etiologies – defect in host defense Innate: IgG deficiency (agammaglobulinemia, subclass deficiency,...) IgA deficiency chronic granulomatous disease (dysf. NADPH oxidase) Acquired AIDS / HIV malnutrition

7. 2. Etiologies – impaired drainage / other Impaired drainage: CF Young’s sy. PCD Kartagener’s sy. Other: RA, Sjoegren’s sy alpha – 1 antitrypsin deficiency GIT disorders (UC, Crohn, GERD) infections in childhood (pertussis, measles, bacterial pneumonia, TBC, adenovirus,...) inhalation of toxic fumes and dusts (NO2, lipoid pneumonia, acids,...) Kartagener’s sy.

8. 3. Clinical findings 1.cough and mucopurulent sputum - months / years 2.dyspnea, wheezing, chest pain 3.recurrent “bronchitis” and frequent antibiotic courses Cough98% Daily sputum78% Rhinosinusitis73% Dyspnea62% Hemoptysis27% Pleurisy20% Crackles75% Wheezing22% Digital clubbing2% *King PT et al. Respir Med 2006; 100: 2183.

9. 4. Diagnosis The purpose of evaluation: 1.radiographic confirmation 2.potentially treatable causes? 3.functional assessment Evaluation: history / examination laboratory testing radiographic imaging pulmonary function testing other testing

10. 4. Diagnosis – laboratory testing 1.CBC, differential BC 2.immunoglobulin quantitation (levels of IgG, IgM, IgA) 3.sputum culture (bact. / TBC / fungi)

11. 4. Diagnosis - CXR dilated airways thickened airway walls irregular periph. opacities (mucus)

12. 4. Diagnosis – Chest CT dilated bronchi bronchial wall thickening “tree – in – bud” pattern cysts lack of tapering

13. Cylindrical bronchiectasis 4. Diagnosis – Chest CT

14. Varicose bronchiectasis 4. Diagnosis – Chest CT

15. Cystis / saccular bronchiectasis 4. Diagnosis – Chest CT

16. Traction bronchiectasis (fibrosis) 4. Diagnosis – Chest CT

17. 4. Diagnosis - distribution 1.central (perihilar) – ABPA 2.predominant upper lobe – CF, Young sy, post - TBC 3.middle /lower lobe – PCD 4.lower lobe – “idiopathic”

18. 4. Diagnosis - distribution Post – TBC bronchiectasis with aspergilosis

19. 4. Diagnosis – lung function FEV1– low FVC– normal or low TI– low (obstruction) hiperresponsive ness– often present

20. 4. Diagnosis – other tests bronchial biopsy (ciliary ultrastructure) bronchoscopy – obstructing lesion? aspergillus precipitins / antibodies serum IgE Ig subclasses alpha 1 – antitrypsin (concentracion / phenotype) RF....

21. 5. Summary 1.clinical findings (cough & sputum) 2.radiographic confirmation 3.identification of treatable causes 4.functional assessment are important for proper treatment plan.

22. P.S. – have you known...... that the largest subgroup represent elderly women. The prevalence of urinary incontinence is 47%, compared with 10 – 12% in general population. * Prys-Picard CO, Niven R. Urinary incontinence in patients with bronchiectasis. Eur Respir J 2006; 27: 866 - 7.

23. Thank you. University Clinic Golnik, Slovenia