FLASHPATHH a z e m A l i

BRONCHIECTASIS

H a z e m A l i

CLINICAL

Bronchiectasis is one of the “obstructive lung diseases” that include:• Chronic bronchitis

• Emphysema

• Small-airway disease “bronchiolitis”

• Asthma

CLINICALObstructive airway

diseaseRestrictive airway

diseaseGeneral features Increase in resistance to

airflow due to obstruction at any level

Reduced expansion of lung parenchyma

Total lung capacity (TLC)

Increased Reduced

Forced Expiratory Volume in one second (FEV1)

Reduced Normal

CLINICAL• Bronchiectasis is permanent dilation and destruction of the

airways– 2ry to recurrent infections and bronchial obstruction– Mainly affect bronchi and bronchioles proximal to the terminal

bronchioles

• Usually present with:– Chronic, productive cough

• Copious amount of foul smelling, purulent sputum– Dyspnea– Fever– Others: of the original cause / associated complications

CLINICAL• High-resolution CT is the procedure of choice for noninvasive

diagnosis– Bronchiectasis is radiologically classified into:

• Cylindrical• Saccular• Cystic

• Effective antibiotic therapy has led to a marked decrease in the incidence of bronchiectasis and its complications:

– Bronchopleural fistula with empyema– Brain abscess– 2ry (systemic) Amyloidosis

CLINICALCauses of

bronchiectasis

Congenital Necrotizing Pneumonia

Bronchial Obstructio

nInflammatory Disorders Idiopathic

• Cystic fibrosis• Intralobar

sequestration• Primary ciliary

dyskinesia• Kartagener syndromes

• Bacteria• Staph,

Strept• Pseudomona

s• TB

• Viruses• Pertussis• Measles

• Fungi

• True obstruction:• Tumor• Foreign bodies• Mucus

impaction• Functional

obstruction:• COPD

• RA• SLE• IBD• Transplant

rejection

CLINICAL• Cystic fibrosis is commonly associated with Bronchiectasis, due to:

– Obstruction (mucus plugs)– Infection (decreased ciliary clearance of bacteria)  

• Defective ciliary function (dyskinesia) is also associated with Bronchiectasis, e.g. Kartegener syndrome:

– Autosomal recessive– Absent or irregular dynein arms (motor part) of cilia– Defective bacterial clearance (bronchiectasis, sinusitis)– Defective cell motility during embryogenesis (situs inversus)– Defective sperm movement (male infertility)

CLINICAL

• Aspergilloma (fungal ball) can occur within pulmonary cavities (e.g. prior TB, Bronchiectasis, old Infarcts, or lung Abscesses). 

• Allergic bronchopulmonary aspergillosis (occurs with asthma and cystic fibrosis) may lead to proximal bronchiectasis and fibrotic lung disease

PATHOGENESISAccumulation of Secretions

Secondary Infection

Inflammation,Necrosis,Fibrosis

Permanent Dilatation of

bronchi

More Accumulation of Secretions

Bronchial Obstruction

GROSS• Mainly noticed at the lower lobes

• Bilateral in 50% of cases

• Bronchi are markedly dilated– Diameter of the bronchus exceeds the diameterof the accompanying bronchial artery– Can form cystic spaces

• Bronchi are filled with yellowish-greenish secretions

GROSS• Sometimes the dilated bronchi can extend out to the

pleural surface– Normally, the bronchioles cannot be followed by eye beyond a

point 2 to 3 cm from the pleural surfaces

Also other features of Cause/Complications:• Pneumonia• Lung abscesses• Empyema• Aspergillosis• Tumor• Foreign body

MICROSCOPYAirways:• Bronchial Architecture:

– Dilated (may be cystic)– Mucopurulent exudate

• Mucosa:– Erosion/Ulceration– Squamous metaplasia

• Wall:– Chronic Inflammation (+/- prominent lymphoid follicles)– Destruction of cartilage / muscle / submucosal glands– Fibrosis

Also other features of Cause/Complications:• Pneumonia• Lung abscesses• Empyema• Aspergillosis• Tumor• Foreign body

CYTOLOGY

In long-standing bronchiectasis you get atypical squamous metaplasia and isolated bizarre squamous cells

– You need numerous bizarre cells to diagnose SCC with confidence

DIFFERENTIAL DIAGNOSIS

Chronic bronchitis

Bronchiectasis Asthma

Small-airway disease

“bronchiolitis”

Emphysema

Site L a r g e a i r w a y s ( B r o n c h i ) Bronchioles AlveoliMajor

pathology

• Mucous gland hyperplasia

• Excess mucus

• Inflammation

• Airway dilation & scarring

• Thickened basement membrane

• Smooth muscle hyperplasia

• Excess mucus

• Inflammation(eosinophils)

• Inflammatory scarring & obliteration

• Airspace enlargement

• Wall destruction

• No fibrosis

Other obstructive lung diseases:

DIFFERENTIAL DIAGNOSISOther causes of chronic cough• Lung carcinoma• Bronchiectasis• Cystic fibrosis• Congestive heart failure• Tuberculosis

DIFFERENTIAL DIAGNOSISO t h e r c o n g e n i t a l / c y s t i c l u n g d i s e a s e s :• Congenital:

– Bronchogenic cysts– Congenital pulmonary cysts– Congenital pulmonary airway malformation– Congenital lobar emphysema– Pulmonary sequestration

• Acquired:– Healed abscess– Honeycombing– Emphysema

DIFFERENTIAL DIAGNOSISMiddle lobe syndrome:• Chronic or recurrent atelectasis (radiographic opacification) of the right

middle lobe, lingula, or both• Mainly due to bronchial obstruction:

– Enlarged lymph nodes– Tumor

• Combinations of the following features:– Atelectasis– Bronchiectasis– Chronic inflammation (+/- Lymphoid hyperplasia)– Organizing pneumonia– Abscesses– Granulomatous inflammation

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