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