clinical manifestation and diagnosis of bronchiectasis aleš rozman university clinic of respiratory...
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Clinical manifestation and diagnosis of bronchiectasis
Aleš RozmanUniversity 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:
1.airway obstruction
2.defect in host defence
3.impaired drainage
4.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
1. cough and mucopurulent sputum - months / years
2. dyspnea, wheezing, chest pain
3. recurrent “bronchitis” and frequent antibiotic courses
Cough 98%
Daily sputum 78%
Rhinosinusitis 73%
Dyspnea 62%
Hemoptysis 27%
Pleurisy 20%
Crackles 75%
Wheezing 22%
Digital clubbing 2%
*King PT et al. Respir Med 2006; 100: 2183.
4. Diagnosis
The purpose of evaluation:1. radiographic confirmation2. potentially treatable causes?3. functional assessment
Evaluation:• history / examination• laboratory testing• radiographic imaging• pulmonary function testing• other testing
4. Diagnosis – laboratory testing
1. CBC, differential BC
2. immunoglobulin quantitation (levels of IgG, IgM, IgA)
3. sputum culture (bact. / TBC / fungi)
4. Diagnosis - CXR
dilated airwaysthickened airway walls
irregular periph. opacities (mucus)
4. Diagnosis – Chest CT
dilated bronchi
bronchial wall thickening
“tree – in – bud” pattern
cysts
lack of tapering
Cylindrical bronchiectasis
4. Diagnosis – Chest CT
Varicose bronchiectasis
4. Diagnosis – Chest CT
Cystis / saccular bronchiectasis
4. Diagnosis – Chest CT
Traction bronchiectasis (fibrosis)
4. Diagnosis – Chest CT
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”
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
1. clinical findings (cough & sputum)2. radiographic confirmation3. identification of treatable causes4. 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.
Thank you.University Clinic Golnik,
Slovenia