Bronchiectasis Clinical Notes

Bronchiectasis: an irreversible airway dilation, which may be either focal or diffuse.

Classically, it has been categorized as cylindrical or tubular (the most common form), varicose, or cystic.

v  ETIOLOGY:

   -          Can be infectious or non-infectious.

   -          Focal Bronchiectasis: bronchiectatic changes in a localized area of the lung as a consequence of obstruction of the airway; which can be extrinsic (e.g. lymphadenopathy or tumor) or intrinsic (e.g. airway tumor or aspirated foreign body).

   -          Diffuse Bronchiectasis: widespread bronchiectatic changes throughout the lung and often arises from an underlying systemic or infectious disease process.

Area of Lung	Causes of Bronchiectasis
Upper Lobe Bronchiectasis	-          Cystic Fibrosis -          Post Radiation Fibrosis
Lover Lobe Bronchiectasis	-          Chronic recurrent aspiration -          End-stage fibrotic lung disease -          Recurrent immunodeficiency state (Hypogammaglobulinemia)
Mid-field Bronchiectasis	-          Mycobacterium avium intracellular complex (MAC) -          Congenital: Immobile cilia syndrome
Central airway involvement	-          Allergic Broncho-Pulmonary Aspergillosis (ABPA) -          Congenital: Tracheobronchomegaly, William Campbell syndrome.

v  Pathogenesis ND Pathology:

Most accepted mechanism of infectious bronchiectasis: “vicious cycle hypothesis”.

   -          Traction bronchiectasis: Dilated airways rising from parenchymal distortion as a result of lung fibrosis (e.g.: Post-radiation fibrosis).

v  CLINICAL MANIFESTATIONS:

Most common presentation: Productive cough with thick tenacious sputum.

Auscultation: Crackles and/or wheeze.

Clubbing may be present.

v  Diagnosis:

   -          Chest X-Ray: Less sensitive for diagnosis. The presence of “Tram tracks” indicates dilated airways.

   -          CT chest: “Tram track” sign or “signet ring sign”, lack of bronchial tapering, bronchial wall thickening, inspissated secretions (e.g. tree in bud pattern) or cysts in bronchial wall.

v  Treatment:

   -          ANTIBIOTIC TREATMENT:

o   Antibiotics targeting the presumptive pathogen (Haemophilus influenza and P. aeruginosa isolated commonly) should be administered in acute exacerbations, usually for a minimum of 7–10 days.

o   Non-Tb Mycobacterium (NTM) should be treated when: Two positive sputum culture or 1 BAL fluid sample positive on culture or NTM proved in biopsy along with 1 sputum positive culture.
Treatment includes a Macrolid combined with rifampicin and ethambutol.

   -          BRONCHIAL HYGIENE:

o   Hydration and use of Mucolytic agents.

o   Chest physiotherapy (e.g. postural drainage).

o   Mucolytic dornase (DNase): in Cystic Fibrosis related bronchiectasis.

   -          ANTI-INFLAMMATORY THERAPY:

o   Glucocorticoids: May be used as oral or parenteral route.

o   Important in certain aetiologies like ABPA or non-infectious bronchiectasis.

v  PREVENTION:

   -          Smoking abstinence.

   -          Reversal of immunodeficient state (e.g. by administration of gamma globulin).

   -          Vaccination of chronic patients (e.g. influenza and pneumococcal infections).

   -          Recurrences (i.e. 3 or more episodes per year) prevention: use of suppressive antibiotics.

o   It includes:
1. Administration of oral antibiotics like ciprofloxacin daily for 1-2 weeks per month.
2. Use of rotating use of antibiotics (to prevent resistance).
3. Administration of macrolide antibiotics daily or three times per week.
4. Inhalational aerosolized antibiotics.
5. Intermittent use of IV antibiotics.