These lecture notes were prepared by Dr. Hamdi Turkey- Pulmonologist- Department of internal medicine - Taiz university Do Not Forget To Visit Our Pages On Facebook on the following Links: https://www.facebook.com/groups/569435236444761/ AND https://www.facebook.com/groups/690331650977113/

1. Bronchiectasis
HamdiAl-Turkey-chest physician

2. Objectives
To know diseases that are associated with BR.
To have a general idea about the major clinical features.
To know how to treat various causes of bronchiectasis.
Identify key differences in the treatment between BR
and CF.

3. Definition
Bronchiectasis (broncos, airways; ectasia,
dilatation) is a morphological term used to describe
abnormal irreversibly dilated and often thick-
walled bronchi.
This is an anatomic definition and is thought to have
evolved from Laennec’s original description in 1819 of
ectatic bronchi in pathological specimens. Bronchiectasis
represents the end stage of a variety of pathologic
processes that cause destruction of the bronchial wall and
its surrounding supporting tissues.

4. History
Described initially in the
early 19
th
century By Laënnec
in patients with chronic
suppurative phlegm.
“This affection of the
bronchia is always produced
by chronic catarrh, or by
some other disease attended
by long, violent, and often
repeated fits of coughing”.
R.T.H. Laënnec.

5. Sir William Osler
“Dilation of the bronchi occurs …
As a congenital defect or
anomaly. Such cases are
extremely rare…
In connection with
inflammation of the
bronchi, particularly when
this leads to weakness of
the walls with the
accumulation of secretion.”

6. Prevalence
Bronchiectasis was a common disabling and fatal condition in the
pre-antibiotic era. It remains an important cause of suppurative
lung disease in the developing world. More recently, the declining
incidence of this disease in the developed world has led to
repeated suggestions that it be considered an orphan disease.
In the United States, the prevalence has recently been
estimated to be 52 per 100,000.
A slight female preponderance has been suggested

7. Pathology
bronchial dilatation is associated with destructive and
inflammatory changes in the walls of medium-sized airways,
often at the level of segmental or subsegmental bronchi
Airway inflammation is primarily mediated by neutrophils and
results in up-regulation of enzymes such as elastase and matrix
metalloproteinases
The normal structural components of the wall, including
cartilage, muscle, and elastic tissue, are destroyed and may be
replaced by fibrous tissue.

8. Pathology
The dilated airways frequently contain pools of thick, purulent
material, while more peripheral airways are often occluded by
secretions or obliterated and replaced by fibrous tissue
Additional microscopic features include bronchial and
peribronchial inflammation and fibrosis, ulceration of the
bronchial wall, squamous metaplasia, and mucous gland
hyperplasia
The parenchyma normally supplied by the affected airways is
abnormal, containing varying combinations of fibrosis,
emphysema, bronchopneumonia, and atelectasis

9. Pathology
As a result of inflammation,
vascularity of the bronchial
wall increases, with
associated enlargement of
the bronchial arteries and
anastomoses between
bronchial & pulmonary
arterial circulations

10. Epithelial injury
Mucus hypersecrtion
Reduced mucociliary
clearance
Plugging of the
airways
InflammationChronic bronchial
infection
Airway damage
Bronchiectasis
Mucus secretogogus
Ciliotoxin
Reactive oxygen species
Proteinase enzymes
IL-8
TNF-alpha
LTB4
Reactive oxygen species
Elastase
MMPs

11. Post infective
• Severe pneumonia
• Tuberculosis
• Pertussis
• Measles
Impaired mucociliary clearance
• CF
• Primary ciliary dyskinesia
• Young’s syndrome
Immunodeficiency
• Common variable immunodeficiency
• Specific polysaccharide antibody deficiency
Secondary immunodeficiency,eg,
malignancy(chronic lymphocytic leukemia)or
human immunodeficiency virus infection
Exaggerated immune response
• Allergic broncho pulmonary aspergillosis
• Graft versus host disease
• Inflammatory bowel disease (ulcerative colitis
and Crohn’s disease) .
Congenital abnormalities of the bronchial wall
• Mounier-Kuhn syndrome
• Williams-Campbellsyndrome
• Marfan syndrome
Inflammatory pneumonitis
• Aspiration of gastric contents
• Smoke inhalation
Fibrosis(traction bronchiectasis)
• Sarcoidosis
• Idiopathic pulmonary fibrosis
Mechanical obstruction
• Foreign body
• Tumor
• Extrinsic compression (eg,lymph node)
Miscellaneous conditions
• Primary Mycobacterium avium complex
infection(“Lady Windermere syndrome”)
Connective tissue diseases
• rheumatoid arthritis
• systemic lupus erythematosus
• Sjo¨gren syndrome
Pulmonary sequestration
Yellow nail syndrome
Infertility(primary ciliary dyskinesia,cystic
fibrosis,Young syndrome)
Diffuse pan bronchiolitis
a1-Antitrypsin deficiency

13. What is the current relevance of previous severe lower
respiratory tract infections to patients with bronchiectasis?
• Bronchiectasis may be the sequela of a variety of
necrotizing infections that are either inadequately
treated or not treated at all.
• Primary infection (ie, in the absence of intrinsic
defects or noninfectious extrinsic insults) was a
particularly common cause of bronchiectasis in
developed countries prior to the widespread use of
antibiotics and it remains important in developing
countries, where antibiotics are used inconsistently.

14. Klebsiella species
Staphylococcus aureus
Mycobacterium tuberculosis
Mycoplasma pneumoniae
Nontuberculous mycobacteria
Measles virus
Pertussis virus
Influenza virus
Herpes simplex virus
Certain types of adenovirus
Typical infectious agents that have been
shown to cause Bx

15. Focal post-obstructive bronchiectasis may occur in a number
of clinical settings (eg, endobronchial tumors,
broncholithiasis, bronchial stenosis from infections,
encroachment of hilar lymph nodes, foreign body aspiration).
Right-middle lobe syndrome is a specific type of bronchial
obstruction that may result in bronchiectasis. It results from
an abnormal angulation of the lobar bronchus at its origin,
predisposing it to obstruction, subsequent infection, and
development of bronchiectasis.
Bronchial obstruction

16. In adults, foreign body aspiration often takes place in the setting
of altered mental status and involves unchewed food. Patients
may also aspirate chewed materials from the stomach, including
food, peptic acid, and microorganisms.
After aspiration, a post-obstructive pneumonia may occur, with
subsequent development of focal bronchiectasis. Bronchiectasis
may also develop in the setting of chronic aspiration. Further
recognized is that a history of gastroesophageal reflux is a risk
factor for aspiration and that the organism Helicobacter pylori
may play a role in the development of bronchiectasis in this
group of patients.
Aspiration

17. Immunodeficiency states may be congenital or acquired. The most common
congenital conditions (albeit rare) involve B-lymphocyte functions.
Hypogammaglobulinemia in these cases may take one of the following
forms :
• Immunoglobulin G (IgG) subclass deficiency
• X-linked agammaglobulinemia
• Immunoglobulin A (IgA) deficiency
• Immunoglobulin M (IgM) deficiency
• Immunoglobulin E (IgE) deficiency
Patients with hypogammaglobulinemia usually present in childhood with
repeated sinus or pulmonary infections, although the disorder has been
diagnosed in adults who did not have a history of repeated infections.
Establishing the diagnosis is important because gammaglobulin replacement
may reduce the number of infections and resultant lung injury.
Immune deficiency and Bx

18. ►The possibility of underlying immune deficiency, particularly antibody deficiency,
should be considered in all children and adults with bronchiectasis.
►Serious, persistent or recurrent infections, particularly involving multiple sites, or
infections with opportunist organisms should raise the suspicion of immune
deficiency.
►The possibility of symptomatic or clinically silent bronchiectasis should be
considered as a potential complication in all patients with immune deficiency,
particularly primary antibody deficiency.
►In patients with immune deficiency and patients with bronchiectasis, features in the
history or clinical examination which may support the coexistence of both conditions
should be considered and adequately assessed.
►Humoral immunodeficiency syndromes involving deficiencies of IgG, IgM, and IgA
can cause recurrent suppurative sinopulmonary infections and BR.
Immune deficiency and Bx

19. CF is a multisystem disorder that affects the chloride transport system in
exocrine tissues, primarily secondary to a defect in the CF
transmembrane regulator (CFTR) protein. CF and its variants are the
most common cause of bronchiectasis in the United States and other
industrialized nations.
CF is an autosomal recessive disease affecting approximately 1 in 2,500
whites and 1 in 17,000 blacks in the United States.
The major pulmonary finding in CF is bronchiectasis, which is an
almost universal feature of this disease. It may be the sole feature of CF
in adults or those with genetic variations of the disease. Bronchiectasis
associated with CF is believed to occur secondary to mucous plugging
of proximal airways and chronic pulmonary infection, especially with
mucoid P aeruginosa.
Cystic Fibrosis (CF)

20. One of these plus one of these
≥1 typical phenotypic
features of CF
Elevated sweat chloride
test on 2 occasions
Sibling with CF
2 identified CFTR
mutations
Positive newborn
screening test (IRT)
Abnormal nasal
potential difference (at
research centers)
Dr Hamdi Turkey
Diagnostic criteria for CF

21. Mutant CFTR gene in CF

22. Autosomal recessive inheritance

24. Julia and CF

25. Young syndrome is clinically similar to CF and may
represent a genetic variant of CF. It is most often observed in
middle-aged men in North America and is a leading cause of
male infertility.
Patients with Young syndrome have bronchiectasis (often
predominant in the lower lobes), sinusitis, and obstructive
azoospermia. However, they do not display the other
findings of CF. The pathogenesis of bronchiectasis in these
patients is believed to be similar to that of bronchiectasis in
CF. The criterion standard for diagnosis of Young syndrome
is electron microscopic analysis of the structure of the cilia.
Young syndrome

26. Primary ciliary dyskinesia is a group of inherited disorders that may affect 1 in
15,000-30,000 population. It is manifested by immotile or dyskinetic cilia and/or
sperm.
PCD is a genetically heterogeneous disorder affecting motile cilia which are made up of
approximately 250 proteins. Around 90%of individuals with PCD have ultrastructural
defects affecting protein(s) in the outer and/or inner dynein arms which give cilia their
motility, with roughly 38% of these defects caused by mutations on two genes, DNAI1
and DNAH5, both of which code for proteins found in the ciliary outer dynein arm.
• The main consequence of impaired ciliary function is reduced or absent mucus
clearance from the lungs, and susceptibility to chronic recurrent respiratory infections,
including sinusitis, bronchitis, pneumonia, and otitis media. Progressive damage to the
respiratory system is common, including progressive bronchiectasis beginning in early
childhood, and sinus disease (sometimes becoming severe in adults)
• diagnosis is often missed early in life despite the characteristic signs and symptoms. In
males, immotility of sperm can lead to infertility
Primary ciliary dyskinesia

27. Schematic diagram of the eukaryotic cilium. Cross-section illustrates the 9 + 2 configuration
of nine peripheral microtubular doublets surrounding a central pair microtubule complex.
The expanded view of a microtubular doublet schematically depicts crosssections of the
tubulin protofilaments including those shared by the A and B tubules. The dynein arms in the
expanded view are rendered to schematically depict several light, intermediate, and heavy
chains comprising each of these structures. Although the outer arm exhibits a specific
distribution of dyneins, being uniformly composed of three heavy, two intermediate and at
least eight light chains, the distribution of dyneins in the inner arm is thought to be more
variable.

28. Electron micrographs of nasal cilia from patients with primary ciliary
dyskinesia (PCD) illustrating dynein defects. Far left panel illustrates
ultrastructure of a normal cilium from nasal epithelium of a healthy,
clinically unaffected subject. The adjacent panels from three different
patients with PCD illustrate defects in both dynein arms, isolated defects
of outer dynein arms only, and isolated defects of inner dynein arms only.

29. Kartagener syndrome is a subset of primary ciliary dyskinesia, an
autosomal recessive condition characterized by abnormal ciliary
structure and/or function leading impaired mucociliary clearance.
situs inversus
chronic sinusitis and/or nasal polyposis
bronchiectasis
Kartagener syndrome is characterised by the clinical triad of:
Kartegner syndrome

30. Kartegner syndrome

31. • Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity
reaction to inhaled Aspergillus antigen that is characterized by
bronchospasm, bronchiectasis, and immunologic evidence of a
reaction to Aspergillus species.
• ABPA should be suspected in patients with a productive cough who
also have a long history of asthma-type symptoms that do not respond
to conventional therapy.
• Bronchiectasis is believed to be secondary to airway plugging by viscid
secretions containing hyphae of Aspergillus species. The resulting
bronchiectasis is thin-walled and affects the central and medium-sized
airways.
Allergic bronchopulmonary aspergillosis

32. Dr Hamdi Turkey
Diagnostic criteria for ABPA

33. Diagnostic criteria for ABPA

34. Bronchiectasis can result from a variety of congenital anatomic defects :
Bronchopulmonary sequestration is a congenital abnormality classified as either
intralobar or extralobar and results in chronic lower respiratory tract infections that
lead to bronchiectasis.
Williams-Campbell syndrome (congenital cartilage deficiency) is the absence of
cartilage from lobar to first- to second-generation segmental airways that results in
extensive peripheral bronchiectasis.
Mounier-Kuhn syndrome (tracheobronchomegaly) is a rare disorder characterized by
dilation of the trachea and segmental bronchi (central bronchiectasis).
Swyer-James syndrome (unilateral hyperlucent lung) likely is a developmental
disturbance that leads to unilateral bronchiolitis, hyperinflation, and, in some cases,
bronchiectasis.
Yellow-nail syndrome is rare. It results in exudative pleural effusions.
Congenital anatomical defects

35. This patient had a 20-year history of severe
lymphedema of her legs; thick, ridged,
yellowish, hypercurved thumbnails (top
right); similarly affected, yellow-green to
brown toenails (bottom right); and bilateral,
chylous pleural effusions. A sample of her
chylous pleural fluid is shown to the left of
the radiograph. This syndrome of yellow
nails, lymphedema, and pleural effusions
(usually serous) presumably results from
defective lymphatic drainage. Women are
affected almost twice as much as men, and
prolonged survival is the rule.
Yellow nail syndrome

36. Clinical features

37. Symptoms of bronchiectasis
Cough
Chronic productive cough is prominent,occurring in up to 98% of patients. Sputum
is typically produced on a daily basis in greater than 70% of patients, Some patients
produce sputum only with acute upper respiratory tract infections, but otherwise they
have quiescent disease.
Sputum is typically mucoid and relatively odorless. During infectious exacerbations,
however, sputum becomes purulent and may develop an offensive odor.
In the past, total daily sputum amount has been used to characterize the severity of
bronchiectasis, with less than 10 mL defined as mild bronchiectasis, 10-150 mL
defined as moderate bronchiectasis, and greater than 150 mL defined as severe
bronchiectasis. dry bronchiectasis manifests as episodic hemoptysis with little-to-no
sputum production. Dry bronchiectasis is usually a sequela of tuberculosis.
Hemoptysis
Hemoptysis occurs in 56-92% of patients with bronchiectasis. Hemoptysis is more
commonly observed in dry bronchiectasis. Hemoptysis is generally mild and
manifested by blood flecks in the patient's usual purulent sputum. Bleeding usually
originates from dilated bronchial arteries, which contain blood at systemic pressures.
Therefore, massive hemoptysis may occur but is rarely a cause of death.
Dyspnea
Dyspnea may occur in as many as 72% of patients; a 2006 review reported a
rate of 62%.
Dyspnea typically occurs in patients with extensive bronchiectasis observed on
chest radiographs.

38. Symptoms of bronchiectasis
Wheezing
Wheezing is commonly reported and may be due to airflow obstruction
following destruction of the bronchial tree. Similar to dyspnea, it may also
be secondary to concomitant conditions such as asthma.
Chest pain
Pleuritic chest pain is an intermittent finding, occurring in 19-46% of
patients. It is most commonly secondary to chronic coughing but also occurs
in the setting of acute exacerbation.
Fatigue
Fatigue is commonly reported (73% of patients). Weight loss often occurs in
patients with severe bronchiectasis. This is believed to be secondary to
increased caloric requirements associated with the increased work of
coughing and clearing secretions.
Fever Fever may occur in the setting of acute infectious exacerbations.
Urinary
incontinence
Urinary incontinence occurs more frequently in women with bronchiectasis
versus age-matched controls (47% vs 12%).The etiology of this is unclear.

39. Physical signs
Wheeze
Scattered wheezing may be heard in approximately one third
of patients; wheezing may be due to airflow obstruction from
secretions, destruction of the bronchial tree leading to airway
collapsibility, or a concomitant condition.
Crackles
Crackles and rhonchi are often observed in association
with active infections and acute exacerbations
Clubbing
Digital clubbing is an inconsistent finding in
approximately 2-3% of patients ; it is more frequent in
patients with moderate-to-severe bronchiectasis
Signs of cor
pulmonale
Right-sided heart failure may be observed, including
peripheral edema, hepatomegaly, and hypoxia

41. Bronchiectasis as a result of infection generally involves the lower lobes,
the right-middle lobe, and the lingula
Right-middle lobe involvement alone suggests right-middle lobe
syndrome, an anatomic dysfunction, or a neoplastic cause with
secondary mechanical obstruction
Bronchiectasis caused by cystic fibrosis (CF), Mycobacterium
tuberculosis infection , or chronic fungal infections tends to affect the
upper lobes, although this is not universal in CF
Allergic bronchopulmonary aspergillosis (ABPA) also affects the upper
lobes but usually involves the central bronchi, whereas most other
forms of bronchiectasis involve distal bronchial segments
The anatomical distribution of bronchiectasis may
be important in helping diagnose any associated
condition or cause of bronchiectasis

42. Investigations

43. sputum analysis
A sputum analysis may reinforce the diagnosis of
bronchiectasis and add significant information regarding
potential etiologies. Once sputum is allowed to settle, the
examination may reveal Dittrich plugs, small white or
yellow concretions. A Gram stain and culture result may
reveal evidence of microorganisms, including mucoid
Pseudomonas species and Escherichia coli, which suggest CF
but are not diagnostic.
Chronic bronchial infection with nonmucoid Pseudomonas
aeruginosa is becoming much more common in patients
with non-CF bronchiectasis. The presence of eosinophils and
golden plugs containing hyphae suggests Aspergillus species,
although this finding alone is not diagnostic of ABPA.
Perform a smear and culture of sputum for mycobacteria
and fungi. Atypical mycobacterial infection is a common
cause of bronchiectasis in the older population, especially in
those with underlying structural lung disease.
Consider suppurative lung disease when
you encounter three layered sputum
(a purulent sediment, clear middle liquid
and a top foamy layer) with foul smell.

44. Laboratory investigations
CBC
The CBC is often abnormal in patients with bronchiectasis. Typical findings
are nonspecific and include anemia and an elevated white blood cell count
with an increased percentage of neutrophils. An increased percentage of
eosinophils is one criterion for ABPA. Alternatively, polycythemia secondary
to chronic hypoxia may be observed in advanced cases.
immunoglobulin
level
including IgG subclasses, IgM, and IgA, are useful to exclude
hypogammaglobulinemia. Note, however, that on rare occasions, bronchiectasis
may be seen in patients with antibody production deficiency but normal to low-
normal IgG levels. In situations such as these, evaluating antibody response to
Haemophilus influenzae and pneumococcal vaccines may be useful.
Alpha1-
Antitrypsin
Quantitative serum alpha1-antitrypsin (AAT) levels are used to rule out AAT
deficiency. In addition to a suggestive family history, clinical features of
emphysema that suggest the possibility of AAT deficiency and the need for serum
testing include onset at an early age (45 y or less) and the absence of a
recognized risk factor (eg, smoking, occupational dust exposure).
Sweat test
Pilocarpine iontophoresis (sweat test) was the criterion standard test to
evaluate for CF. However, genetic analysis has now become standard and
may be performed to look for evidence of mutations consistent with CF
and to look for potential variants, such as Young syndrome.

45. Lab. Investigations (2)
Aspergillus
Precipitins and
Serum Total IgE
levels
Aspergillus precipitins and serum total IgE levels are important in
making the diagnosis of ABPA. Diagnostic criteria for ABPA
include a total serum IgE level greater than 1000 IU/mL or a
greater than 2-fold rise from baseline.
Autoimmune
Screening
Rheumatoid factor and/or other screening tests for autoimmune
disease may be performed in the appropriate clinical setting. For
example, an antinuclear antibody (ANA) assay may also be
considered.
Electron
Microscopic
Examination
Perform electron microscopic examination of sperm and
respiratory epithelium to observe for evidence of primary ciliary
structural abnormalities and dyskinesia. These will be found in
disorders such as primary ciliary dyskinesia.
Spirometry
Pulmonary function test results may be normal or abnormal.
The most common abnormality is an obstructive airway defect,
which may even be found in patients without a prior smoking
history.

46. CXR
Posterior-anterior and lateral chest
radiographs should be obtained in all
patients. Expected general findings
include increased pulmonary markings,
honeycombing, atelectasis, and pleural
changes. Specific findings may include
linear lucencies and parallel markings
radiating from the hila (tram tracking) in
cylindrical bronchiectasis, dilated bronchi
in varicose bronchiectasis, and clustered
cysts in cystic bronchiectasis. In the
appropriate clinical setting, chest
radiograph findings are occasionally
sufficient for confirming the diagnosis of
bronchiectasis.

47. HRCT of the chest
CT scanning, particularly high-resolution CT (HRCT) scanning of the chest, has
replaced bronchography as the defining modality of bronchiectasis. CT sensitivity and
specificity reportedly are 84-97% and 82-99%, respectively, but may be higher at referral
centers.
The following are noteworthy aspects of CT findings in bronchiectasis:
Cylindrical bronchiectasis has parallel tram track lines, or it may have a signet-ring
appearance composed of a dilated bronchus cut in a horizontal section with an adjacent
pulmonary artery representing the stone
The diameter of the bronchus lumen is normally 1-1.5 times that of the adjacent vessel; a
diameter greater than 1.5 times that of the adjacent vessel is suggestive of bronchiectasis
Varicose bronchiectasis has irregular or beaded bronchi, with alternating areas of
dilatation and constriction
Cystic bronchiectasis has large cystic spaces and a honeycomb appearance; this contrasts
with the blebs of emphysema, which have thinner walls and are not accompanied by
proximal airway abnormalities

48. Cylindrical bronchiectasis. A, B, Examples
from two patients. Airways parallel to the
plane of section in the anterior segment of an
upper lobe show changes of cylindrical
bronchiectasis; bronchi are wider than normal
and fail to taper as they proceed towards the
lung periphery.
Varicose bronchiectasis. Patient
with allergic bronchopulmonary
aspergillosis and cystic fibrosis.
The bronchiectatic airways have a
c o r r u g a t e d , o r b e a d e d ,
appearance.
Cylindrical bronchiectasis Varicose bronchiectasis

49. Cystic bronchiectasis in the upper lobes in two patients. In such advanced
disease, it is often impossible to distinguish between markedly dilated
bronchi and cystic airspaces in destroyed lung. A, Etiology of the
bronchiectasis was unknown in this patient. B, Patient had cystic fibrosis.
Cystic bronchiectasis

50. Management

51. General approach and treatment
of the specific underlying cause
►Identify and treat underlying cause to prevent disease progression.
►Maintain or improve pulmonary function.
►Reduce exacerbations.
►Improve quality of life by reducing daily symptoms and exacerbations.
►In children, achieve normal growth and development.
►Patients with primary or secondary immune deficiency should be
under joint care with a clinical immunologist.
►Patients with CF should be referred to a CF specialist centre.

52. The following general measures are recommended:
Smoking cessation
Avoidance of second-hand smoke
Adequate nutritional intake with supplementation, if necessary
Immunizations for influenza and pneumococcal pneumonia.
Confirmation of immunizations for measles, rubeola, and pertussis
Oxygen therapy is reserved for patients who are hypoxemic with
severe disease and end-stage complications, such as cor pulmonale.
Supportive treatment

53. Physiotherapy: airway clearance
techniques and exercise
Good bronchial hygiene is paramount in the treatment of bronchiectasis, because of the
tenacious sputum and defects in clearance of mucus in these patients. Postural drainage
with percussion and vibration is used to loosen and mobilize secretions.
Devices available to assist with mucus clearance include flutter devices, intrapulmonic
percussive ventilation devices, and incentive spirometry.Although consistent benefits
from these techniques are lacking and vary with patient motivation and knowledge, a
review did report improvement in patients’ cough-related quality of life scores.
Nebulization with concentrated (7%) sodium chloride solutions appears to be beneficial,
particularly in patients with CF-related bronchiectasis. Mucolytics, such as acetylcysteine,
are also often tried but do not appear to be universally beneficial. However, maintaining
adequate general hydration, which may improve the viscidity of secretions, is important.
Aerosolized recombinant DNase has been shown to benefit patients with CF. This enzyme
breaks down DNA released by neutrophils, which accumulates in the airways in response
to chronic bacterial infection. However, improvement has not been definitively shown in
patients with bronchiectasis from other causes.

54. Chest physiotherapy (CPT)
Chest physiotherapy (CPT) is a set of techniques that include
percussion, vibration, and postural drainage. The purpose of CPT is
to loosen respiratory secretions and move them into the central
airways where they can be removed by coughing or suctioning.
Removing secretions from the airway and not allowing them to
accumulate reduces the risk for respiratory infections and atelectasis.
CPT is often performed along with other therapies such as
bronchodilators, antibiotics, mucolytic agents, and hydration.
Combing these therapies helps reduce mucous production and
increase airway clearance.

55. Chest percussion
Percussion involves striking the skin over congested
lung fields to dislodge secretions from the bronchial
walls. It can be accomplished using cupped hands or
commercial devices such as percussion cups. When
you use your hands to percuss, hold your fingers and
thumb together and flex them slightly to form a cup.
When your cupped hand contacts the area to be
percussed, the air is trapped against the chest,
propelling vibrations through the chest wall to the
secretions.
When performing percussion, cover the area you are
percussing with a towel or the patient’s gown to
reduce any discomfort. Instruct the patient to breathe
slowly and deeply and, in an alternating manner, flex
and extend your wrists rapidly to percuss the chest.
Percuss each area of the lung that is congested for 1 to
2 minutes or as prescribed. Avoid percussion over the
breasts, sternum, spinal column, and kidneys.

56. Vibration
Vibration is used after percussion or
alternately with percussion to increase the
turbulence of exhaled air and loosen
secretions. To perform vibration, place your
hands on the affected area either side by side
or with one hand on top of the other and
your fingers extended and together. While
the patient exhales slowly through pursed
lips or the nose, tense your hand and arm
muscles and move the heel of your hand in a
shaking manner to create vibrations through
the patient’s chest. When the patient inhales,
stop moving your hands. Perform vibration
over the affected area during five
exhalations, or as prescribed. After each
vibration, instruct the patient to cough.

57. Postural drainage
Postural drainage is performed to remove secretions by gravity
from different areas of the lungs. To drain the affected areas,
place the patient in a variety of positions to facilitate drainage
by gravity. Not all positions are required for every patient and,
depending on the patient’s illness or condition, some positions
may be contraindicated.
Postural drainage is commonly performed two or three times a
day, often before meals and at bedtime. It is best to schedule it
when the patient’s stomach is empty to avoid gastric reflux and
vomiting. If the patient is receiving continuous tube feedings,
stop the feeding and check gastric residual at least 30 minutes
before performing postural drainage.
Before starting postural drainage and during the procedure,
evaluate the patient’s tolerance of the various positions. The
patient usually remains in each position for 10 to 15 minutes.
However, this time may be shorter initially and then gradually
increased as the patient is better able to tolerate it.
When performing postural drainage, first position the patient,
then percuss and vibrate, then remove the secretions either by
having the patient cough or by suctioning the patient’s airway.

58. Antibiotics
• Antibiotics are given during the acute exacerbation
( empiric with amoxicillin 500 mg tid for 7-10 days or
other antibiotics like ciproflxacin 500 mg bid for 7-10
days and other alternatives according to sputum culture
•Patients with bronchiectasis are at increased risk of
frequent infectious exacerbations.
• Prevention of these Exacerbations is thought to be
achieved by decreasing the “Bacterial Load” in the airways.
•Several strategies have been tried to minimize the risk of
these exacerbations.

59. Preventive antibiotic therapy
• Daily oral antibiotic treatment, such as Ciprofloxacin 500 to
1500 mg/day, given in 2 to 3 divided doses, given for seven
to 14 days of each month .
• Erythromycin for 8 weeks
A double-blind placebo-controlled study.
To evaluate the effects of 8-week administration of low dose
erythromycin (
Patients in the erythromycin group had significantly
improved forced expiratory volume in one second, forced
vital capacity and 24-h sputum volume after 8 weeks
(p<0.05)
• Daily or three times weekly use of azithromycin (250mg)

60. • Aerosolization of an antibiotic
• Tobramycin at a dose of 300 mg BID every
other month for six months in patients with CF
had a 100-fold reduction in sputum
Pseudomonas density, improved FEV1, and
decreased hospitalizations compared to patients
receiving placebo aerosol
Preventive antibiotic therapy

61. IV antibiotics
• Intermittent IV antibiotics should be reserved for
patients with resistant organisms (such as
Pseudomonas) or in preparation for major
surgery, including resection of a bronchiectatic
region of lung and other procedures during
which pulmonary function may be compromised.

62. Pseudomonas
• Almost impossible to eradicate in patients
with bronchiectasis.
• Patients have reduced quality of life indices,
more extensive bronchiectasis on CT, and
increased number of hospitalizations.

63. MAC
• MAC is often harbored in damaged lung
tissue and bronchiectatic airways.
• ATS recommends treatment until the
patient is culture negative for 12 months.
• A combination of azithromycine/
clarithromycine + rafimpicin+
ethumbatol

64. ABPA
• Oral Prednisone (0.5-1.0 mg/kg/day) is
the cornerstone of therapy for patients
with allergic bronchopulmonary
aspergillosis.
• Patients may also benefit from additional
therapy with a prolonged course of
Itraconazole (400 mg/day)

65. Bronchodilators
• Airway reactivity, presumably due to
transmural inflammation, is often present
in patients with bronchiectasis
• Aerosol bronchodilator therapy, may be
appropriate but has not been studied in
patients with bronchiectasis

66. Anti-inflammatory drugs
Macrolide antibiotics
Inhaled corticosteroids

67. Surgery
Indications:
Removal of focal bronchiectasis with destroyed lung
~ Removal of destroyed lung partially obstructed by a tumor
or the residue of a foreign body
~ Reduction in overwhelming purulent and viscid sputum
production
~ Elimination of bronchiectatic airways subject to
uncontrolled hemorrhage
~ Removal of an area suspected of harboring resistant
organisms such as MAC.