BRONCHIECTASIS

1. BRONCHIECTASIS -
ETIOPATHOGENESIS

2. • word derived from Greek , bronchion
meaning windpipe and ektasis
stretching out
• bronchiectasis is present when one or
more bronchi are abnormally and
irreversibily dilated
• not a separate disease but a result of
various affections of lungs and bronchi

3. PATHOGENIC MECHANISMS
AND AETIOLOGY

4. Primary infective insult : Bronchitis/bronchiolitis
Pertussis
Measles
Adenovirus
Pneumonia
Tuberculosis
Primary impairment of mucous clearance : Cystic fibrosis
Primary ciliary
dyskinesias

5. Immunodeficiency syndromes: Common varied immunodeficiency
Selective immunoglobulin deficiency
Functional immune deficiency
Secondary hypogammaglobulinaemia
Human immunodeficiency virus infection
• Hyperimmune responses: Allergic bronchopulmonary mycoses

6. Autoimmune disease : Inflammatory bowel disease
Coeliac disease
Systemic lupus erythematosus
Rheumatoid disease
Cryptogenic fibrosing alveolitis
Primary biliary cirrhosis
Thyroiditis
Pernicious anaemia
Inhalational/aspiration injury: Toxic fumes
Gastric contents

7. Developmental defects
Structural Biochemical :
Pulmonary agenesis Antitrypsin deficiency
Sequestrated segment
Tracheobronchomegaly
Bronchomalacia

8. Infection secondary to bronchial
Obstruction
intraluminal extraluminal
slow growing tumour lymphadenopathy
aspirated foreign body

9. SYNDROMES OF CILIARY
DYSKINESIS

10. abnormalities of ciliary function
DEFECTS : absence of one or both rows of dynein arms
OR
absence of spoke heads or central sheaths in
others
CLINICAL FEATURES:
• Male infertility
• Dextrocardia
• Respiratory symptoms ( from infancy or early childhood) 
chronic rhinorrhoea and sinusitis
• otitis media
• mild deafness
• cough

11. KARTAGENER’S SYNDROME
Triad of
chronic sinusitis
Bronchiectasis
Situs inversus

12. IMMOTILE CILIA SYNDROME
• Primary ciliary dyskinesis
• transmitted by autosomal recessive gene with
incomplete penetrance

13. YOUNG’S SYNDROME
• Idiopathic obstructive azoospermia
• chronic paranasal sinusitis
• lower respiratory tract disease
• obstructive azoospermia
• due to a common defect of ciliated columnar epithelium at these
different sites

14. • history of mercury intoxication in childhood (‘pink disease’,
caused by mercurous chloride in teething powders and
vermifuges) was obtained in some patients who later
developed Young’s syndrome,

15. CYSTIC FIBROSIS
• hereditary disorder
• activity of a chloride channel - cystic fibrosis transmembrane
conductance regulator (CFTR) is reduced due to genetic mutation
• early symptoms are due to pancreatic insufficiency
• patients who survive develop persistent lower respiratory tract
infection  bronchiectasis

16. lungs of patients with CF are virtually normal in utero, but once
breathing commences
Electrolyte transport defect
desiccated and viscid secretions
impair mucociliary clearance
trapped organisms lead to a cycle of persistent and
increasing inflammatory load
widespread bronchiectatic changes

17. Classic triad :
pulmonary disease
pancreatic insufficiency
raised sweat sodium concentration of 70mmol/L or more
azoospermic as a result of congenital bilateral absence of vas
deferens

18. INHALATIONAL INJURY
• developing as a consequence of inhalation or aspiration
of toxic or irritant substances, either in liquid form or
when contained in smoke or fumes

19. DEVELOPMENTAL DEFECTS

20. ALPHA 1 ANTITRYPSIN DEFICIENCY
• proteases released from phagocytes during pyogenic infection
may cause bronchial wall damage if unopposed by
antiproteases (a1-antitrypsin )

21. PULMONARY AGENESIS
• most bronchi in a lobe or lung are found to be dilated
• present since childhood with symptoms and signs consistent
with bronchiectasis.
• affected part of the lung shows no evidence of alveoli
• partial pulmonary agenesis with a failure of peripheral parts of
lung to develop.

22. PULMONARY SEQUESTRATED SEGMENT
• Bronchiectasis may occur congenitally
within an intralobar sequestration that
contains disorganized, dilated and
deformed bronchi
• These may communicate with normal
surrounding lung tissue if sequestration
becomes complicated by infection and
ruptures.

23. TRACHEOBRONCHOMEGALY
• Mountier Kuhn syndrome
• Congenital
• cartilagenous rings of the trachea and its divisions as far as segmental
bronchi are enlarged
• marked dilatation of the trachea and central bronchi
• elastic and muscular tissues between rings of cartilage are atrophic
and may tend to bulge between rings in manner of tracheal
diverticulae

24. • intrathoracic trachea and main
bronchi dilate during inspiration
and collapse on expiration
• associated with chronic lower
respiratory tract infection and
bronchiectasis
• diagnosed in 4th or 5th decades

25. WILLIAMS CAMPBELL SYNDROME
• Bronchomalacia
• congenital syndrome
• defective or completely absent bronchial wall cartilage 
producing mechanical abnormality  bronchiectasis
• Symptoms ( cough) begin from infancy.
• defect may extend from the 4th to the 8th generations of
bronchi
• CT shows ballooning expansion of the proximal bronchi
during inspiration, with collapse during expiration

26. IMMUNODEFICIENCY
SYNDROMES

27. AGAMAGLOBULINAEMIA
•congenital X-linked (Bruton’s) agammaglobulinaemia,
•producing infection in infancy once maternal IgG is
exhausted

28. COMMON VARIED IMMUNODEFICIENCY
(CVID)
• Presenting at any age
• peak incidence in childhood and adolescence
• associated with chronic sinusitis and repeated episodes of infective bronchitis leading to
bronchiectasis in adult life.
• levels of immunoglobulins are rather variable
• IgG reduced to less than 2g/L
• IgA is often virtually undetectable
• IgM may be reduced to less than 0.2g/L

29. • selective IgG subclass deficiencies -- may be found in the presence of a
normal total IgG level
• Bronchiectasis is not usually associated with isolated IgA or IgM
deficiencies but is more likely if these occur in association with selective
IgG subclass deficiencies

30. FUNCTIONAL IMMUNOGLOBULIN DEFICIENCY
• These patients fail to produce antibodies in response to a specific challenge

31. • Immunity also be impaired by both neutrophil and T-cell
dysfunction.
• Bronchiectasis also been associated with a natural killer cell
dysfunction in which lymphocytes do not express human leucocyte
antigen (HLA) class I antigen on their surface  bare lymphocyte
syndrome’

32. HUMAN IMMUNODEFICIENCY VIRUS
human immunodeficiency virus infection
repeated infection - common respiratory pathogens, such as
Streptococcus pneumoniae, Haemophilus influenzae, Moraxella
catarrhalis,Staphylococcus aureus and Pseudomonas
aeruginosa, pneumocystic carinii
bronchiectasis

33. INFECTIONS

34. PERTUSSIS
• WHOOPING COUGH
• Bordetella pertussis
• spread by droplet infection
• Infection
elaboration of local toxins
ciliostasis with inflammation and oedema of the tracheal
and bronchial mucosa necrosis with sloughing of cells.

35. MEASLES
• severe inflammation of bronchial wall
• Most important complication pneumonia

36. ADENOVIRUS
• account for about 5% of respiratory infections in children
• serotypes 1, 3, 4, 7 and 21 more virulent than others
•MYCOBACTERIUM

37. PNEUMONIA
• When pneumonia precedes bronchiectasis, acute event has
often occurred in childhood and is poorly documented
• pneumonic episode may follow on the heels of epidemic
childhood infection
• Bacterial pneumonic infections are much more likely to be
controlled  widespread availability of antibiotics

38. OBSTRUCTION
Bronchiectasis result from
(i) obstruction of many small peripheral bronchi
(ii) obstruction of a more central bronchus

39. Proximal and prolonged more central bronchial obstruction may
result from
(i) intraluminal occlusion
slow-growing tumour – carcinoid tumours , benign
tumour ( lipoma, papilloma, fibroma, chondroma)
aspirated solid foreign body
(ii)extramural compression -- mediastinal or other masses

40. • Obstruction of small peripheral bronchi is a feature of LRTI in
children, notably where mucus and inflammatory debris are shed
into lumina of peripheral bronchi (because of their small
diameters)

41. MIDDLE LOBE SYNDROME
• Brock’s syndrome
• middle lobe bronchus susceptible to occlusion because of its
relatively small lumen and its emergence from intermediate
bronchus at a right angle
• this point surrounded by lymph nodes that if enlarged tend to
compress it

42. ASPIRATION OF FOREIGN BODY
• right side is affected more often than left
• usually lower lobe or posterior segment of the upper lobe.

43. AUTOIMMUNE DISEASES

44. AUTOIMMUNE DISEASES
Autoimmune diseases associated with bronchiectasis :
inflammatory bowel disease,
ulcerative colitis
coeliac disease
primary biliary cirrhosis
rheumatoid arthritis
systemic lupus erythematosus
thyroiditis
pernicious anemia

45. • lymphocyte-mediated defect resulting in failure to
recognize ‘self’, with consequent production of
antibodies against a range of normal tissue
components, including mucosal surfaces

46. HYPERIMMUNE RESPONSE

47. ALLERGIC BRONCHOPULMONARY MYCOSES
• Atopic subjects, who usually have asthma, are affected
by this condition which arises as a result of allergy to
Aspergillus spp
• A. fumigatus frequently seen.
• CXR :‘fleeting infiltrates’ (or rounded opacities)
• fleeting shadows clear spontaneously or with systemic
corticosteroid treatment
• pathology shows an alveolar eosinophilic infiltrate
• central bronchiectasis
Chest radiograph
showing rounded
opacities

48. YELLOW NAIL SYNDROME
triad : yellow, thickened, dystrophic finger-nails
chronic dependent lymphoedema
pleural effusions
result from hypoplastic peripheral and pleural lymphatics,
associated with sinusitis, recurrent pulmonary infections and
bronchiectasis.

49. MECHANISMS OF BRONCHI DILATION
1. ATELECTASIS THEORY
2. PRESSURE OF SECRETION THEORY
3. TRACTION THEORY

50. ATELECTASIS THEORY
• Terminal bronchioles subtending acini (primary lobules) are
obstructed by secretions.
• Air is absorbed from acini which collapse,creating increased
negative pressure on bronchi.
• bronchi dilate into saccules.

51. PRESSURE AND SECRETION THEORY
• plugging of a bronchus with mucus or other material
• secretions distal to obstruction accumulate and mechanically
distend the bronchi beyond block

52. TRACTION THEORY
• bronchial dilatation occurs secondarily to fibrosis of lung
parenchyma, the resulting scar tissue requiring high inflation
pressures on inspiration to overcome abnormally high retractive
forces

53. CLASSIFICATION
1. FOLLICULAR BRONCHIECTASIS
2. SACCULAR BRONCHIECTASIS
3. ATELECTATIC BRONCHIECTASIS

54. FOLLICULAR BRONCHECTASIS
• presence of numerous lymphoid follicles situated in thickened,
usually cylindrically dilated bronchial walls

55. SACCULAR BRONCHIECTASIS
• presence of macroscopically visible thin-walled, saccular
(sometimes called cystic) bronchial dilatations

56. ATELECTATICS BRONCHIECTASIS
• associated with pulmonary collapse

57. REID’S CLASSIFICATION
• Depending on findings of CT scan
• 1.cylindrical bronchiectasis
• 2.varicose bronchiectasis
• 3.cystic bronchiectasis

58. REFERENCE
• CROFTON AND DOUGLAS’S RESPIRATORY DISEASES

59. THANK YOU