BRONCHIECTASIS

Bronchiectasis : Destruction / Dilatation of large airways - airways are filled with pus results in the features of bronchorrhea

HISTOPATHOLOGICAL  EXAMINATION:

CYLINDRICAL / TUBULAR - MOST COMMON subtype - Represents airways are dilated and thick walled which filled with pus by the dangerous organisms like pseudomonas. this organism later will form the biofilm in the lungs which results in antibiotic resistant. expansion of pus filled cavity will erode the blood vessels of lungs which results is massive hemoptysis and death of the patient.

Cause of  death - Massive hemoptysis

Causes :

A) FOCAL - Obstruction which subsequently cause infection in the airway and results in pus. following condition can cause focal bronchiectasis

- Foreign body aspiration (children)

- Pulmonary hamartoma (adults)

- Bronchial carcinoid (adults)

note: Benign tumor in adults like pulmonary hamartoma and carcinoid tumors which encroached the bronchus and result in obstruction of airways and causes atelectasis. so the secretion of lungs become

infective in atelectasis area.

B) DIFFUSE - 

(A) INFECTION :

- Pseudomonas Aeruginosa (affects mucociliary clearance)

- Mycoplasma Pneumoniae (affect mucociliary clearance)

(Resistant to beta lactams so macrolides should given)

- Bordetella Pertusis (100 days illness)

(B) α1 Antitrypsin deficiency (genetic) :

It is produced by liver but the problem here is to release of  α 1 antitrypsin from the liver. so main function of α 1 antitrypsin is neutralization of elastase from macrophages. This function is impaired in this condition.

EFFECTS:

a) Panacinar Emphysema

b) Bronchiectasis

c) Cirrhosis

(C) Cystic fibrosis (genetic)

- Autosomal Recessive

- F508 mutation, defect in chromosome 7

- Mucus become thick and viscid, so there is stasis of mucus

- infertility seen in both male and female

(D) Kartagener syndrome (genetic)

Immotile cilia due to dynein arm defect

TRIAD:

a) Recurrent sinusitis

b) Bronchiectasis

c) situs inversus

INFERTILITY - Not a part of triad

(E) Decreased immunity 

- HIV

- Hypogammaglobulinemia

(F) Autoimmunity

-a) Rheumatoid Arthritis, sjorgen syndrome , inflammatory bowel disease

- RA  can develop inflammatory nodules which results in local airway damage and later it may leads to fibrosis which can distort the anatomy of the airway

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-b) ABPA (Allergic Bronchopulmonary Aspergilloma ) - it is not colonized by aspergillosis species but it can  cause hypersensitivity reaction to fungus ASPERGILLUS FUMIGATUS and results in local damage of lungs

- Predisposing factor here is asthmatic where patient with brown to black sputum plugs

- so diagnosis is made based on :

CT  scan for central bronchiectasis - present with middle lobe involvement

Serum precipitins against aspergillus - checking for antibodies against aspergillus which can damage the middle lobe of the lungs

(G) Recurrent Aspiration (Micro aspiration)

a) Scleroderma (esophageal dysmotility) - aspiration of food particles taking place when patients is lying down

b) Bulbar palsy - Palsy seen with stroke patients where vagus nuclei involved

CLINICAL FEATURES:

• Productive  Cough
• Bronchorrhea
• Dyspnea on exertion
• Crept present
• Clubbing - present because long duration of illness

 

WORK UP :

• CHEST X- RAY : Mostly present with normal, in few cases presents with TRAM TRACK appearance on lower lobe
• CT - scan (HRCT) - Investigation of choice

ON HRCT :

• TRAM TRACK appearance

• Signet ring

• Tree in bud

• PULMONARY FUNCTION TEST - Obstructive pattern

• SPUTUM CULTURE

• HIV - TO TEST WITH ELISA

• GENETIC STUDY - CFTR gene

• α1 Antitrypsin assay

• NASAL / LUNG BIOPSY - Test for kartagener syndrome 

• VIDEOMICROSCOPY OF LUNG (CILIA)

• RA FACTOR

TREATMENT :

1. Antibiotics  x 14 days 
2. Non TB Mycobacterium - Macrolide + Rifampicin or Ethambutol
3. Mucolytic - Dornase , DNAase
4. Percussion device (helps in dislodgement of mucus) - chest physiotherapy , Incentive spirometry
5. Bronchial hygiene

COMPLICATION :

Massive Hemoptysis (500ml to 600ml/day)

IOC - Rigid Bronchoscopy

TOC - Bronchial Artery Embolization