Respiratory system

1. DR NASIR FAROOQ BUTT
ASSOCIATE PROFESSOR
DEPARTMENT OF MEDICINE
KING EDWARD MEDICAL UNIVERSITY
WEST MEDICAL WARD
MAYO HOSPITAL LAHORE

2. Mr. Nazir, 28 years, male, day laborer, smoker,
presented with frequent cough with profuse
expectoration of mucoid sputum for 2 years, marked
in the morning. Occasionally, the sputum was
purulent and foul smelling, relieved after taking
antibiotics. He also noticed small amount of blood
occasionally mixed with sputum. For the last 6
months, he also complained of about 5 kg weight
loss, weakness, malaise, occasional fever and night
sweating. There was no history of chest pain,
breathlessness or swelling feet. His bowel and bladder
habits were normal. There was history of measles
during childhood. He also gives history of recurrent
chest infection for the last 2 years.

3. On Examination
He was emaciated with Clubbing involving toes and fingers.
Pulse: 88/min. BP: 120/75 mm Hg.
Respiratory rate: 30/min.
Exam of Resp. system showed normal vesicular breathing with multiple
coarse crepitations on the bases of both lungs, both in inspiration and
expiration which disappeared/reduced after coughing.
Other systemic examination revealed no abnormalities.
Diagnosis?

5. BROCHIECTASIS
•DEFINITION
•PATHOPHYSIOLOGY
•CAUSES
•CLINICAL FEATURES
•DIAGNOSIS
•MANAGEMENT
•CYSTIC FIBROSIS
•Other Important Causes

6. BROCHIECTASIS- DEFINITION
Derived from the Greek words:
Bronckos – airway
Ectasis – widening
•Bronchiectasis is a chronic
respiratory disease characterized
by a clinical syndrome of cough,
sputum production and bronchial
infection
AND
•Radiologically - It is the abnormal, permanent
dilatation of one or more bronchi with
destruction of bronchial wall proximal to the
terminal bronchiole.

7. BROCHIECTASIS
•Bronchiectasis shares many clinical features with
COPD, including inflamed and easily collapsible
airways, obstruction to airflow, and frequent office
visits and hospitalizations.
•Prevalence increases with age.
•More common in women
•Extensive healthcare resources.

8. BROCHIECTASIS
PATHOLOGY OF BRONCHIECTASIS
•Characteristic feature: multiple bronchiectatic
cavities
•Left lung is involved more than right lung
•Lower lobes are involved more than upper lobes
due to more efficient drainage of upper lobes by
gravity
•Common sites of involvement: lower lobes,
lingula and middle lobe
•Smaller bronchi with less supportive cartilage are
predominantly involved

9. BROCHIECTASIS- PATHOLOGY
• Inflammation. The inflammatory process associated with pulmonary
infection damages the bronchial wall, causing a loss of its supporting
structure and resulting in thick sputum that ultimately obstructs
the bronchi.
• Distention. The walls become permanently distended and distorted,
impairing mucociliary clearance.
• Collapse. The retention of secretions and subsequent obstruction
ultimately cause the alveoli distal to the obstruction collapse.
• Scarring. Inflammatory scarring or fibrosis replaces functioning lung
tissue.
• Symptoms. In time, the patient develops respiratory insufficiency with
reduced vital capacity, decreased ventilation, and an increased ratio of
residual volume to total lung capacity.
• Impairment. There is impairment in the match of ventilation to
perfusion and hypoxemia.

11. BROCHIECTASIS – morphological types
All three forms may be present in the same patient.
1. Cylindircal (Fusiform):
•Commonest form
•Involves airways from 6th-10th generation
•Bronchi have uniform calibre, do not taper and have
parallel walls
2. Varciose:
•Resembles varicose vein
•Beaded appearance where dilated bronchi have
interspersed sites of narrowing
•Relatively uncommon

12. BROCHIECTASIS
3. Saccular (Cystic):
• Occurs in proximal bronchi
• Dilation ends in large cysts, saccules or grape-like clusters
• Most severe form

13. BROCHIECTASIS- CAUSES
A. CONGENITAL:
•Cystic fibrosis
•Kartagener’s syndrome (primary ciliary
dyskinesia or immotile ciliary syndrome,
characterized by bronchiectasis, infertility and
situs inversus)
•Immunodeficiency due to
hypogammaglobulinaemia (may be congenital or
acquired)
•Congenital kyphoscoliosis
•Young’s syndrome (obstructive azoospermia
and chronic sinopulmonary infection, thought to
be due to mercury intoxication)
•Yellow nail syndrome (pleural effusion,
lymphedema, yellow dystrophic nails)

14. BROCHIECTASIS- CAUSES
B. Acquired
• Infections:
Viral: Measles
Bacterial: Pertussis, Tuberculosis (the
commonest cause worldwide)
Fungal: Aspergillosis, Allergic
bronchopulmonary aspergillosis(ABPA).
• Mechanical bronchial obstruction: causing
external compression
By a foreign body
By a mass (e.g. tumor or lymph node)
causing external compression

15. BROCHIECTASIS- CAUSES
B. Acquired
In association with autoimmune disorders:
Rheumatoid arthritis
Inflammatory bowel disease
• Recurrent aspiration:
Gastro-oesophageal reflux disease
Alcoholics

17. BRONCHIACTESIS – Clinical features
• COUGH (98%) WITH SPUTUM (78%):
If localized areas affected - sputum
production vary with position.
As the condition worsens:
persistent halitosis
recurrent febrile episodes with malaise
episodes of pneumonia.
When the condition is severe there is
continuous production of foul-smelling,
thick, khaki-coloured sputum.

18. BRONCHIACTESIS – Clinical features
•Hemoptysis (27%): can occur either as blood-
stained sputum or as a massive hemorrhage.
It is due to bronchial wall hypertrophy, hence mucosa
becomes friable, slough out, capillary opens and bleeding
occurs. Erosion of hypertrophic bronchial artery may result
in massive hemoptysis.
•Breathlessness (62%) may result from airflow
limitation.
•Clubbing (2%)
•Coarse crackles (75%) can be heard over the
infected areas, usually the lung bases.

19. BRONCHIACTESIS – Clinical features
• Rhinosinusitis (30 %)
• Recurrent pleurisy (20 %)
• Wheezing (22 %)
• In more advanced disease, weight loss and
cachexia are prominent
• Bronchiectasis Sicca:
 Dry cough
Associated with intermittent episodes of hemoptysis. It
may be massive, even life-threatening as bleeding is
from bronchial vessels with systemic pressure.
Common in patients with granulomatous infection,
especially TB
Usually involves upper lobe.

20. BRONCHIACTESIS – Clinical features
If the sputum is kept in a bottle, there are three layers:
• Upper frothy layer.
• Middle thick or liquid layer
• Lower sediment (epithelial
debris and bacteria) layer

21. BRONCHIECTASIS– INVESTIGATIONS
•Complete blood count with differential
•Serum immunoglobulins. IgG, IgM, and IgA
levels .Up to 10% of adults with bronchiectasis
have antibody class or subclass deficiency
(mainly IgA).
•Sweat test for sodium and chloride content: If
cystic fibrosis is suspected (sweat chloride
content > 60 mEq/L is suggestive of cystic
fibrosis)

22. BRONCHIECTASIS– INVESTIGATIONS
Pulmonary Function Test: SPIROMETRY
•Usually obstructive but may be normal or show a
restrictive or mixed pattern
•Obstructive impairment
Reduced or normal forced vital capacity [FVC]
Low forced expiratory volume [FEV1]
Low FEV1/FVC is the most frequent finding
•A very low FVC can also be seen in advanced disease
in which much of the lung has been destroyed.

24. BRONCHIECTASIS– INVESTIGATIONS
Chest X-ray:
•May be normal
•Ring shadows – bunch of grapes
•Ring with clear centre (like honeycomb)
•Ring with or without fluid level, may be
multiple (cystic bronchiectasis)
•Linear streaks (tram line)
•Thick bronchi, signet ring or patchy opacity.
Dilated and thickened airways that appear as:
Ring like shadows (of airways that are on end) or
Tram Lines (in the case of airways that are perpendicular to the
x-ray beam)

29. In the lower lobe you can see a cluster of typical ring shadows giving a
‘bunches of grapes’ appearance (1)together with bronchial wall
thickening seen side-on, which gives two thick parallel lines, known as
tramline shadows (2).

30. BRONCHIECTASIS– INVESTIGATIONS
Localization of bronchiectasis according to underlying cause

31. BRONCHIECTASIS– INVESTIGATIONS
CT SCAN CHEST
•Conventional CT scan has sensitivity of 60 to 80%
while High Resolution CT scan (HRCT) has a sensitivity
of more than 90%
•In standard CT scan, the resolution is 10 mm thick. But
in HRCT, resolution is 1 to 2 mm thick.
•Previously bronchography was done.

32. BRONCHIECTASIS– INVESTIGATIONS
High-resolution CT scanning reveals thickened,
dilated bronchi and cysts at the end of the
bronchioles. Characteristically the airways are
larger than their associated blood vessels.
•Signet Ring sign
•Parallel (tram) lines or end-on ring shadows.
•In heavily involved areas, the cysts are clustered
to appear like grapes (cystic bronchiectasis).

37. BRONCHIECTASIS– INVESTIGATIONS
• Image 4

38. BRONCHIECTASIS– INVESTIGATIONS
• Image 5

39. BRONCHIECTASIS– INVESTIGATIONS
•SPUTUM EXAMINATION and culture are
essential for adequate treatment.
The major pathogens are Staph. aureus,
Pseudomonas aeruginosa, H. influenzae and
anaerobes. Other pathogens include Strep.
pneumoniae and Klebsiella pneumoniae.
Aspergillus fumigatus can be isolated from
10% of sputum specimens in cystic fibrosis
Mycobacterium avium-intracellulare
complex (MAI) is being increasingly found.

40. BRONCHIECTASIS– INVESTIGATIONS
•Sinus X-rays. 30% of bronchiectasis patients
also have rhinosinusitis.
•Urine for proteinuria (if amyloidosis is suspected)
•USG of whole abdomen (if situs inversus is
suspected).
•Rheumatoid factor measurements
•Bronchoscopy

41. BRONCHIECTASIS
MANAGEMENT

42. BRONCHIECTASIS– MANAGEMENT
Postural drainage:
Patients must be trained by physiotherapists to
tip themselves so that the affected lobe(s) are
uppermost at least three times daily for 10–20
minutes.
Most patients find that lying over the side of the
bed with head and thorax down is effective.

43. BRONCHIECTASIS– MANAGEMENT
• Antibiotics:
• If a specific bacterial pathogen cannot be isolated, then
empiric oral antibiotic therapy for 10 - 1 4 days is
appropriate .
• Common regimens include
• Amoxicillin or amoxicillin-clavulanate (500 mg every 8
hours),
• Ampicillin or tetracycline (250-500 mg four times daily),
• Trimethoprim-sulfamethoxazole (160/800 mg every 12
hours), or
• Ciprofloxacin (500-750 mg twice daily) .
• Staph. Aureus: Flucloxacillin 500 mg 6-hourly

44. BRONCHIECTASIS– MANAGEMENT
ANTIBIOTICS:
• Pseudomonas Aeruginosa: If the sputum remains yellow or
green despite regular physiotherapy and intermittent
chemotherapy, or if lung function deteriorates despite
treatment with bronchodilators, it is likely that there is
infection with Pseudomonas aeruginosa.
• Parenteral or aerosol chemotherapy at regular 3-
month intervals.
• Ceftazidime 2 g intravenously 8-hourly or by inhalation
(1 g twice daily) has been shown to be effective.
• Ciprofloxacin 750 mg twice daily orally may work in
the short term, but resistance can develop rapidly.
• High sputum levels of some antibiotics, e.g. colistin or
tobramycin, can be achieved by inhalation.

45. BRONCHIECTASIS– MANAGEMENT
Antibiotics:
•Preventive or suppressive treatment:
Prolonged macrolide therapy (azithromycin
500 mg three times a week for 6 months or 250
mg daily for 12 months) has been found to
decrease the frequency of exacerbations
High-dose amoxicillin (3 g/day) or alternating
cycles of the antibiotics can be given orally for 2-4
weeks are also used
Inhaled aerosolized aminoglycosides reduce
colonization by Pseudomonas species, improve
FEV1, and reduce hospitalizations.

46. BRONCHIECTASIS– MANAGEMENT
•Bronchodilators: Bronchodilators are useful in
patients with demonstrable airflow limitation.
•Anti-inflammatory agents: Inhaled or oral
steroids can decrease the rate of progression.
•Surgery:
Indications of surgery: Usually in young patient.
Unilateral and localized to a single lobe or
segment
Severe and recurrent hemoptysis.
Lung or heart-lung transplantation is sometimes
required

47. BRONCHIECTASIS– Complications
•Secondary infection (pneumonia and pleurisy),
common organisms are Staphylococcus aureus,
Haemophilus influenzae and Pseudomonas
aeruginosa
•Lung abscess
•Pleural effusion, empyema or pneumothorax
•Pulmonary hypertension and cor pulmonale
•Respiratory failure
•Amyloidosis (commonly involving spleen or
kidney) in long standing case
•Brain abscess (metastatic cerebral abscess)
•Aspergiloma in the bronchiectatic cavity.

48. BRONCHIECTASIS– Complications
• Pneumonia
• Severe, life-threatening Hemoptysis:
Massive hemoptysis originates from the high-pressure
systemic bronchial arteries. Mortality of 25%.
Treatment:
Bed rest
Tranexamic acid
Antibiotics.
Blood transfusion is given if required.
Urgent fibreoptic bronchoscopy is occasionally necessary
to detect the source of bleeding.
If the haemoptysis does not settle rapidly, the treatment
of choice is bronchial artery embolization. Surgical
resection may be required if embolization fails

49. BRONCHIECTASIS – Prognosis
•The advent of effective antibiotic therapy has
greatly improved the prognosis.
•
•Ultimately, most patients with severe
bronchiectasis will develop respiratory failure
or cor pulmonale
•Those with mild disease have normal life
expectancy.

50. Cystic fibrosis

51. Cystic fibrosis
•One of the commonest life-threatening autosomal
recessive conditions (1:2000 live births) affecting
Caucasians.
•Caused by mutations in the CF transmembrane
conductance regulator (CFTR) gene on chromosome 7
(>800 mutations have now been identified).
•Cl channel defect leads to a combination of defective
chloride secretion and increased sodium absorption
across airway epithelium.
•The changes in the composition of airway surface
liquid predispose the lung to chronic pulmonary
infections and bronchiectasis.

53. Cystic fibrosis – Clinical features

54. Cystic fibrosis – INVESTIGATIONS
• Sweat test: sweat sodium and chloride >60mmol/L; chloride
usually > sodium.
• Genetics: screening for known common CF mutations should be
considered.
• Faecal elastase is a simple and useful screening test for exocrine
pancreatic dysfunction.
• Blood: FBC, U&E, LFT; clotting; vitamin A, D, E levels; annual
glucose tolerance test
• Bacteriology: cough swab, sputum culture.
• Radiology: CXR; hyperinflation; bronchiectasis.
• Abdominal ultrasound: fatty liver; cirrhosis; chronic pancreatitis;
• Spirometry: obstructive defect. Aspergillus serology/skin test
(20% develop ABPA,
• Biochemistry: faecal fat analysis.

55. Cystic fibrosis – MANAGEMENT
•Managed by a multidisciplinary team, eg physician,
GP, physiotherapist, specialist nurse, and dietician,
with attention to psychosocial as well as physical
wellbeing.
•Gene therapy (transfer of CFTR gene using liposome
or adenovirus vectors) is not yet possible.
•Chest: Physiotherapy regularly (postural drainage,
active cycle breathing techniques or forced expiratory
techniques). Antibiotics are given for acute infective
exacerbations and prophylactically (PO or nebulized).
• Mucolytics may be useful (eg DNase, ie Dornase alfa,
2.5mg daily nebulized.
•Bronchodilators.

56. Cystic fibrosis – MANAGEMENT
•Gastrointestinal: Pancreatic enzyme replacement; fat
soluble vitamin supplements (A, D, E, K);
ursodeoxycholic acid for impaired liver function;
cirrhosis may require liver transplantation.
•Other: Treatment of CF-related diabetes; screening for
and treatment of osteoporosis; treatment of arthritis,
sinusitis, and vasculitis; fertility and genetic
counselling.
•Advanced lung disease: Oxygen, diuretics (cor
pulmonale); non-invasive ventilation; lung or
heart/lung transplantation.

57. Cystic fibrosis–Prognosis and screening
•Median survival is now ~ 40 years.
•Genetic screening is available for the 20
commonest mutations and this identifies
85–95% of carriers.
Screening for the carrier state should be
offered to persons or couples with a family
history of CF, together with counselling

59. Kartagener syndrome
•Autosomal recessive GENETIC disorder
•Immotile cilia syndrome - immotile cilia and
immotile spermatozoa
•Primary ciliary dyskinesia (PCD) -
uncoordinated and inefficient movement pattern
•Chronic respiratory infections and sterility in
male patients
•Frequency - 1 case per 32,000 live births
•Situs inversus occurs randomly in half the
patients with primary ciliary dyskinesia

60. Kartagener syndrome
pRESENTATION
• Chronic upper and lower respiratory tract infections
resulting from an ineffective mucociliary mechanism
• Chronic PRODUCTIVE cough with unexplained
respiratory distress.
• Improper drainage of the sinonasal system, this leads to
congestion, rhinorrhea, and chronic middle ear
effusions with possible purulent otorrhea.
• Some male patients present later in life with sterility due
to immotile spermatozoa
• Clinical triad of chronic sinusitis, bronchiectasis, and
situs inversus.

61. Kartagener syndrome
•Only standardized definitive diagnostic tool is
electron microscopy, which is used to visualize
ciliary ultrastructure.
•Semen analysis in postpubescent males may
reveal abnormal sperm motility and ultrastructure.
•Chest radiographs may illustrate bronchial wall
thickening as an early manifestation of chronic
infection, hyperinflation, atelectasis,
bronchiectasis, and situs inversus (in 50% of
patients with primary ciliary dyskinesia).
•High-resolution CT scan of the chest

62. Kartagener syndrome- management
•Continuous clinical follow up
•Biannual clinical visits, which would involve
routine spirometry, sputum culture, and, if
needed, imaging studies
•Antibiotics, intravenous or oral and continuous
or intermittent, are used to treat upper and lower
airway infections
•Obstructive lung disease, if present, should be
treated with inhaled bronchodilators and
aggressive pulmonary toilet.
•Mucolytics may be helpful