Clinical manifestation and diagnosis of bronchiectasis Aleš Rozman University Clinic of Respiratory Diseases and Allergy

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

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.

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

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

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, ...)

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

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.

3. Clinical findings cough and mucopurulent sputum - months / years dyspnea, wheezing, chest pain recurrent “bronchitis” and frequent antibiotic courses *King PT et al. Respir Med 2006; 100: 2183.

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

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

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

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

4. Diagnosis – Chest CT Cylindrical bronchiectasis

4. Diagnosis – Chest CT Varicose bronchiectasis

4. Diagnosis – Chest CT Cystis / saccular bronchiectasis

4. Diagnosis – Chest CT Traction bronchiectasis (fibrosis)

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

4. Diagnosis - distribution Post – TBC bronchiectasis with aspergilosis

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

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

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

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.

University Clinic Golnik, Slovenia Thank you.

Clinical manifestation and diagnosis of bronchiectasis Aleš Rozman University Clinic of Respiratory Diseases and Allergy