Bronchiectasis

By Dr Haris Gul
Senior registrar Rheumatology
Fauji Foundation Hospital Rawalpindi
 Bronchiectasis
• Uncommon disease
• Most often secondary to
infections
• That results in the
abnormal and permanent
distortion of one or more
of the conducting bronchi
or airways
 Bronchiectasis
• Can be categorized as a COPD

• Manifested by airways that are

inflamed and easily collapsible
 Bronchiectasis
• Resulting in air flow obstruction with
SOB, impaired clearance of secretions,
and occasionally hemoptysis

• Severe cases can result in progressive

impairment with respiratory failure
 Bronchiectasis
• Most commonly presents as a focal
process involving a lobe, segment,
or sub-segment

• Far less commonly, it may be a

diffuse process involving both lungs
 Typical offending organisms
• Klebsiella species • Measles virus
• Staphylococcus aureus • Pertussis virus
• Mycobacterium • Influenza virus
tuberculosis • Herpes simplex virus
• Mycoplasma • Certain types of
pneumoniae adenovirus
• Nontuberculous
mycobacteria
 Clinical manifestations:

• Cough and daily mucopurulent sputum

• Hemoptysis
• Dyspnea, pleuritic chest pain, wheezing, fever,
weakness, fatigue, and weight loss
• Rarely, episodic hemoptysis with little to no
sputum production
 Exacerbations from acute bacterial
infections:
• Increased sputum production over baseline
• Increased viscidity of sputum
• A foul odor of the sputum
• Low-grade fever
• Increased constitutional symptoms
• Increased dyspnea, SOB, wheeze or pleuritic
pain
 Findings on physical examination:
• Crackles & rhonchi
• Digital clubbing
• Cyanosis and plethora
• Polycythemia from chronic hypoxia
• Wasting and weight loss
• Nasal polyps and signs of chronic sinusitis
• Cor-pulmonale, in advanced disease
 The diagnosis of bronchiectasis involves the
following:
• A compatible history (eg, daily cough and
purulent sputum production)
• Sputum analysis
• Chest radiography
• HRCT scanning is the standard test for
diagnosis
 Management
• Antibiotics and chest physiotherapy
• Bronchodilators
• Corticosteroid
• Dietary supplementation
• Oxygen
• Hospitalization for severe exacerbations
• Surgical therapies
 Acceptable antibiotic regimens for mild to
moderately ill
• Amoxicillin
• Tetracycline
• Trimethoprim-sulfamethoxazole
• Azithromycin or clarithromycin
• A second-generation cephalosporin
• A fluoroquinolone
 For patients with moderate-to-severe
symptoms
• Parenteral administration may be indicated:
• Gentamicin or tobramycin and
Antipseudomonal penicillin
• 3rdgeneration cephalosporin, or
fluoroquinolone
• Surgical resection an important adjunct for
patients with focal disease
 Cystic fibrosis
•  It is caused by a change, or mutation, in a
gene called CFTR (cystic fibrosis trans-
membrane conductance regulator)
• This gene controls the flow of salt and fluids in
and out of the cells
• If the CFTR gene doesn't work the way it
should, a sticky mucus builds up in the body
 Cystic fibrosis
• A disease of exocrine gland function
• Involves multiple organ systems
• Chiefly results in chronic respiratory infections
& pancreatic enzyme insufficiency
• Pulmonary involvement: 90%
• Principal cause death: End-stage lung disease
• Median age at diagnosis is 6-8 months
 Gastrointestinal (GI) symptoms :
• Meconium ileus • Flatulence, steatorrhea
• Abdominal distention • Recurrent abdominal
• Intestinal obstruction pain
• Increased frequency of • Jaundice
stools • GI bleeding
• Failure to thrive

Meconium ileus is a bowel obstruction that occurs when the meconium in

child's intestine is even thicker and stickier than normal meconium,
creating a blockage in the ileum
 Respiratory symptoms
• Cough
• Recurrent wheezing
• Recurrent pneumonia
• Atypical asthma
• Dyspnea on exertion
• Chest pain
 Genitourinary symptoms 
• Undescended testicles or hydrocele
• Delayed secondary sexual development
• Amenorrhea
 Physical signs
• Depend on the degree • Pulmonary system:
of involvement of Tachypnea, respiratory
various organs distress wheeze or
• Nose: Rhinitis, nasal crackles, increased AP
polyps chest diameter,
clubbing, cyanosis,
hyper-resonant chest on
percussion
 GI tract
• Abdominal distention,
hepatosplenomegaly,
rectal prolapse, dry skin,
cheilosis
 Diagnosis

• Requirements for diagnosis include either +ve

genetic testing or +ve sweat chloride test and
1 of the following:
• Typical COPD
• Documented exocrine pancreatic insufficiency
• Positive family history 
 Imaging studies 
• Radiography (chest, sinus, abdomen)
• CT of the chest (not yet advised as a routine
modality in CF)
• Ultrasonography
• Contrast barium enema
 Additional tests
• Genotyping
• Pulmonary function testing
• Bronchoalveolar lavage
• Sputum microbiology
 Management

• The primary goals of treatment:

• Maintaining lung function as near to
normal as possible by controlling
respiratory infection and clearing
airways of mucus
• Administering nutritional therapy to
maintain adequate growth
• Managing complications
 Mild acute pulmonary exacerbations 

• Increasing the frequency of airway clearance

• Inhaled bronchodilator treatment
• Chest physical therapy and postural drainage
• Increasing the dose of the mucolytic agent
• Use of oral antibiotics 
 Medications used to treat CF
• Pancreatic enzyme supplements
• Multivitamins (including fat-soluble vitamins)
• Mucolytics
• Nebulized, inhaled, oral, or intravenous
antibiotics
• Bronchodilators
• Anti-inflammatory agents
 Medications used to treat CF
• Agents to treat associated conditions or
complications (eg, insulin, bisphosphonates)
• Agents devised to reverse abnormalities in
chloride transport
• Inhaled hypertonic saline
 Surgical therapy
• Respiratory –
Pneumothorax
Massive recurrent or persistent hemoptysis
Nasal polyps
Persistent and chronic sinusitis
• GI –
Meconium ileus
Intussusception
Gastrostomy tube placement for supplemental
feeding
Rectal prolapse
• Lung transplantation is indicated for the
treatment of end-stage lung disease