Bronchiectasis

By Author: Ibrahim Machiwala
Total Articles: 463
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16 year old female house lady came in OPD with the presenting complaints of:

Fever off and on low grade for one year
Productive Cough with copious sputum for one year.
Shortness of breath gradually increase over the period of one year.
Recurrent episodes of allergic rhinitis and sinusitis since childhood.
Not gaining weight

Past medical history:
Took antituberculous therapy two months back but no improvement in symptoms.
History of child hood pneumonia at the age of two years
No history of hospitalization.

Family history:
There was no family history of allergic rhinitis, asthma, eczema in the family.

Socioeconomic history:
Patient belong to middle class family.

General physical examination:
Patient sitting comfortably on chair able to speak full sentences.
VITALS
pulse: 110/min
temp : 100oF
R/R : 20/min
BP : 100/70

On general physical examination there was mild pallor with grade II clubbing.
There was no evidence of cynosis, lymphadnapathy and pedal edema.
...
... Rest of general physical examination was unremarkable.

Examination of chest:
Trachea central
Apex beat was not palpable.
On auscultation bilateral coarse crackles more on right side.
There were wheezing present bilaterally.

Examination of the abdomen:
There was nothing remarkble on inspection.
There was no visceromegaly
Examination of CNS and CVS was unremarkable.

BRONCHIECTASIS:
Bronchiectasis is the term used to describe abnormal dilatation of the bronchi. It is usually acquired but may result from an underlying congenital defect of immune or ciliary function.

CAUSES OF BRONCHIECTASIS
CONGENITAL:
Ciliary dysfunction syndrome
Primary ciliary dyskinesia
Kartegeners syndrome
Young's syndrome

Cystic fibrosis:
Hypogamaglobulinaemia

ACQUIRED-ADULTS:
Suppurative Pneumonia
Primary Tuberculosis
Allergic bronchopulmonary Aspergillosis
Bronchial Tumors

ACQUIRED-CHILDREN:
Penumonia (complicating whooping cough or measles)
Primary tuberclosis
Foreign body

PATHOLOGY:
The bronchiectatic cavities may be lined by granulation tissue, squamous epithelium or normal ciliated epithelium. There may also be inflammatory changes in the deeper layers of the bronchial wall and hypertrophy of the bronchial arteries. Chronic inflammatory and fibrotic changes are usually found in the surrounding lung tissue.

REID'S CLASSIFICATION OF BRONCHIECTASIS:
Cylindrical Bronchiectasis
Cystic Bronchiectasis
Varicose Bronchiectasis.

CLINICAL FEATURES:
SYMPTOMS OF BRONCHIECTASIS
Due to accumulation of pus in dilated bronchi.
Due to inflammatory changes in lung and pleura surrounding dilated bronchi.
Haemoptysis
General health

PHYSICAL FINDINGS:
Findings are non specific and may be attributed to other conditions.
Crackles, rhonchi, wheezing and inspiratory squeaks may be heard on auscultation.
General findings include digital clubbing, cyanosis, plethora, wasting and wt. loss.
Nasal polyps and signs of chronic sinusitis may be present.
In advanced cases, the physical stigmata of cor-pulmonale may be observed.

INVESTIGATIONS:
Haematological Investigations
Blood CBC.
Immunoglobin Levels.
IgE Aspergillous antibodies.
Screening for autoimmune
diseases and rheumatoid factors.
Pilocarpine iontophoresis (sweat testing) is used to detect cystic fibrosis.

Sputum Investigation:
Sputum C/S.
AFB Smear and C/S.
Fungal smear and C/S.

Imaging Studies:
Chest X-ray.
HRCT Thorax.

Procedures
Pulmonary Function Test.
Bronchoscopy.

MANAGEMENT:
Antibiotic therapy
Chest physiotherapy and Postural drainage.
Bronchodilators and corticosteroids.
Surgical treatment.

ANTIBIOTICS:
Antibiotics have been the main stay of treatment for more than 40 years.
Acceptable choices for outpatient who is mild to moderately ill include.
Amoxicillin
Co-trimoxazole
Newer generation macrolides like
Azithromycin.
Quinolone
Second generation cephalosporin
Duration is for 7-10 days

MODERATE TO SEVERE SYMPTOMS:
parenteral aminoglycosides (gentamycin,tobramycin)
antipseudomonal synthetic penicillin
third generation cephalosporins
floroquinolone

INFECTION WITH MAC:
3-4 drug regimen with
clarithromycin
rifampicin
ethambutol
streptomycin
Treatment is continued till the patient
Become culture negative for one year
Some patient with chronic bacterial infection may need regular antibiotic treatment to control the infectious process.
Recently nebulized rout of antibiotic has received more attention.
Currently inhaled tobramycin is used widely in patient with cystic fibrosis.

BRONCHIAL HYGIENE:
Chest physiotherapy and postural drainage
Nebulization with sodium chloride and mucolytics such as acetyl- cystiene.
Aerosolized recombinant DNase is mainly used in patient with CF.

Surgical Care:
surgery should be reserved for patients who have focal disease that is poorly controlled by antibiotics.
Other indications for surgical intervention may include the following:
Reduction of acute infective episodes
Reduction of excessive sputum production
Massive hemoptysis (Alternatively, bronchial artery embolization may be attempted for the control of hemoptysis.)
Foreign body or tumor removal
Consideration in the treatment of MAC or Aspergillus species infections

PROGNOSIS:
Disease is progressive when associated with ciliary dysfunction and cystic fibrosis, and inevitably causes respiratory failure and right ventricular failure. In other patients the prognosis can be relatively good if postural drainage is performed regularly and antibiotics are used judiciously.

PREVENTION:
As bronchiectasis commonly starts in childhood following measles, whooping cough or a primary tuberculous infection, it is essential that these conditions receive adequate prophylaxis and treatment. The early recognition and treatment of bronchial obstruction are also particularly important.