BRONCHIECTASIS

 Disease characterized by permanent dilation of bronchi and

bronchioles caused by destruction of the muscle and elastic
tissue, resulting from or associated with chronic necrotizing
infections.
 BRONCHIECTASIS
(Causes)

• Congenital or hereditary conditions: E.g. Cystic fibrosis and

Kartagener syndromes.

• Postinfectious conditions, Bacterial(Mycobacterium tuberculosis,

Staphylococcus aureus, Haemophilus influenzae, Pseudomonas),
Viruses (adenovirus, influenza virus, human immunodeficiency
virus [HIV]), and Fungi (Aspergillus species).
 BRONCHIECTASIS
(Causes)

• Bronchial obstruction : Due to tumor, foreign bodies, Mucus plug

in asthma.

• Other conditions : Rheumatoid arthritis, Systemic lupus

erythematosus, Inflammatory bowel disease, and post-
transplantation.
 BRONCHIECTASIS
(Pathogenesis)

 Obstruction and infection are the major etiologies.

 Bronchial obstruction impedes normal clearing

So, that infections and inflammation can fester and eventually

cause the tissue destruction

Cause Bronchiectasis
 BRONCHIECTASIS
(Morphology)
Grossly:
 Most commonly occurs in the lower
lobes.
 Airways can dilate upto four times the
normal size.
 Numerous cystically dilated bronchi
(Honey comb appearance)
 These dilated bronchi may be –
A) Cylindrical
B) Fusiform
C) Saccular
 BRONCHIECTASIS
(Morphology)

Microscopic examination:

 Spectrum of mild to necrotizing acute and chronic inflammation of

the larger airways.

 In chronic cases fibrosis occurs in the bronchial walls.

 BRONCHIECTASIS
(Clinical features)
 Cough productive of copious sputum
(often cupful, foul smelling).

 Recurrent infections (Most often P.

aeruginosa)

 Hemoptysis that is sometimes massive.

 Digital clubbing.

 Corpulmonale.
 BRONCHIECTASIS
(Investigations)

Chest radiograph, CT scan finding:

 Crowded bronchial markings extend to the lung periphery
 BRONCHIECTASIS

Kartagener syndrome:
- Autosomal recessive
- Immotile cilia due to defect of dynein arms (Primary ciliary
dyskinesia)
- Characterized clinically by:
a) Bronhiectasis b) chronic sinusitis c) Infertility and d) Situs
inversus.
 BRONCHIECTASIS

Cystic fibrosis: -
 AR
 Defect in CFTR gene (Chromosome 7)
 Defective ATP gated Chloride channel that secretes chloride
in lungs and GI tract, and reabsorbs chloride in sweat glands.