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