Bronchiectasis in India: More questions than answers

B
ronchiectasis is a disease in which
there is permanent enlargement
of parts of the bronchi that are
associated with repeated microbial infections.
It is not uncommon in clinical practice. These
patients suffer for their entire life time and
occasionally manifest with life threatening
hemoptysis or lung infection. Appropriate
pharmacotherapy, counselling and regular
physiotherapy need to be given early on and
maintained throughout life. Unfortunately,
we have very little information about
bronchiectasis because this a very poorly
researched area. There are a few hospital/
clinic based observational studies that have
reported several patients of bronchiectasis
and many case studies about bronchiectasis
in India.
The European Lung White Book says that
Asia has the highest prevalence of this disease.
Our anecdotal reports from India, especially
E d i t o r i a l
Dr Raja Dhar
MD, MRCP, MSc, CCT, FCCP
Consultant Pulmonologist Intensivist & Coordinator
Department of Pulmonary & Critical Care Medicine
Fortis Hospital, Kolkata
from Tertiary Care centers, also indicate
a high prevalence in our country. But this
information is clearly not sufficient to help us
guide appropriate measures to either measure,
prevent or treat this disease better. There is
no good data on bronchiectasis in India The
main cause for Bronchiectasis in our country
is likely to be Pulmonary TB.
This is because the largest global burden
of TB is in India. Post-TB bronchiectasis
patients have marked different clinical
features and prognosis, compared with
non-TB bronchiectasis. Keeping in mind
the paucity of Bronchiectasis data,
the EMBARC (European Multicentre
Bronchiectasis Audit and Research
Collaboration) network was established
in 2012 to facilitate multidisciplinary
collaborative research in non-Cystic Fibrosis
bronchiectasis. The aim of the EMBARC
group is :
◆ To create the EMBARC registry, a European
bronchiectasis registry to facilitate research
and quality improvement initiatives across
healthcare systems.
◆ To build a network of researchers and
clinical experts in bronchiectasis to guide
future research and clinical priorities.
◆ To attract new researchers and clinicians
to the field of bronchiectasis.
◆ To support and encourage early career
researchers in the field of bronchiectasis
through involvement in network activities.
◆ To facilitate applications to industry
and European Union funding sources to
build bronchiectasis research capacity in
Europe.
Inspired by this initiative I approached the
EMBARC Steering Committee to build the
EMBARC INDIA Registry under the auspices
of the Respiratory Research Network of
India. The infectious enthusiasm of Dr James
Chalmers, Lecturer in Respiratory Medicine,
University of Dundee, UK facilitated setting
up this Registry in India. The untiring efforts
of Dr Sneha Limaye and Dr Nikita Jalan
helped in setting up a network of 38 centers
some of whom have started recruiting and
we have already recruited about 75 patients
in the last 6 weeks. We hope to present some
of this work at the European Respiratory
Congress in Sept 2016. If you are interested
in taking part in this study, please send
an email to embarc@rrni.net. We would
be more than happy to have your participation
in EMBARC India.

About the Book

While much progress has been made with tuberculosis (TB) control, the
WHO estimates that 9 million people developed TB in 2013, and 1.5 million
died of TB. India accounts for 25% of the global TB burden, and for a third
of the ‘missing cases’ that do not get diagnosed or notified. The emergence of
severe forms of drug-resistance has further complicated the picture. More than
50% of India’s TB patients seek care in the private sector, and private providers
and GPs are often the first point of care even for patients treated in the public
sector. Unfortunately, private practitioners rarely adhere to national and
international standards, and there is plenty of evidence that quality of TB care is
often suboptimal. This results in emergence of drug-resistance, and also explains
the high TB mortality rate in India. Let’s Talk TB is a compilation of a series
of articles published in GP Clinics over the past two years on the current best
practices on TB diagnosis and treatment available for free for all GPs and private
practitioners in India.
Editor, Madhukar Pai,
MD, PhD McGill International TB Center,
McGill University, Montreal, Canada

|Volume V, Issue V, November - December 2015| 1

 Types and pathophysiology of Bronchiectasis
Dr. Priyanka Ghosh
MD, DNB (Pulmonary)
Tutor Dept. of Pulmonary Medicine
Sagore Dutta Medical College, Kolkata
Bronchectasis is a disease characterized
by permanent dilation of bronchi and
bronchioles caused by destruction of
muscle and elastic tissue, resulting from
or associated with chronic necrotizing
infection
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 was a common
disabling and fatal condition in the pre-
antibiotic era. Still 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 attributed to the advent
of improved living conditions, frequent
and early use of antibiotics, improved
sanitation and nutrition and introduction
of childhood immunization, particularly
against measles and pertussis. Prevalence
figures have varied from 4 to 272 per lakh
population, partly dependent upon the
age range studied. There are sparse data
on the prevalence of bronchiectasis in the
Indian subcontinent.
Types of Bronchiectasis
Bronchiectasis may be classified by
predisposing factors, pathological features
and radiographic appearance. Reid in
1950 described a correlation between
pathological and bronchographic findings
in bronchiectasis. Since then this has
been the most widely used classification,
though there is considerable overlap and
coexistence among the various forms. congenital disease or in association with
The first type, cylindrical
bronchiectasis (tubular), is characterized
by uniform dilatation of bronchi, which
extends into the lung periphery, without
tapering. The second type is called varicose
bronchiectasis (illusion to varicose veins)
is characterized by irregular and beaded
outline of bronchi, with alternating
areas of constriction and dilatation. The
third type is called cystic or saccular
bronchiectasis and is the most severe form
of the disease. The bronchi dilate, forming
large cysts (appears as clusters of grapes),
which are usually filled with air and fluid.
However, the clinical usefulness of
designating bronchiectasis to one of these
2 RespiMirror|Volume V, Issue V, November - December 2015|
Rama Saha
MD (Pathology)
Associate Professor
Dept of Pathology, IPGMER, Kolkata
patterns is questionable and no study to
date has shown a clinical, epidemiologic,
or pathophysiologic difference between
these patterns. Traction bronchiectasis
is a different entity, seen in diffuse
pulmonary fibrosis secondary to
fibrous tissue traction and elevated
negative intrathoracic pressure; it is
distinguished because of lack of intrinsic
airway pathology and paucity of sputum
expectoration.
Bronchiectasis can present as either
local disease, or a diffuse process involving
both lungs. Focal bronchiectasis may be
the result of blockage of the bronchial
lumen by a foreign body, tumour or as
a result of extrinsic compression of the
bronchi. The middle lobe syndrome is an
example of focal bronchiectasis caused
by extrinsic compression of the bronchi,
by enlarged lymph node secondary to
mycobacterial or fungal infection. Diffuse
bronchiectasis is usually caused either by
systemic diseases.
Causes of Bronchiectasis
Although many patients seem to have
no associated disease that lead to the
development of bronchiectasis, there
are many conditions that have been
recognized to cause bronchiectasis.
A number of pulmonary infections
have been associated with the development
of bronchiectasis. The association of
measles with bronchiectasis has been
considered a complication secondary to
the intense bronchial and peribronchial
inflammation and epithelial proliferation.
Complicating secondary infections with
Jaydip Deb
MD (Pulmonary) Professor & HOD
Dept. of Pulmonary Medicine, Bankura
Sammilani Medical College, West Bengal
adenovirus, herpesvirus, and bacteria
such as Staphylococcus aureus, Klebsiella
pneumoniae, and P. aeruginosa possibly
further contribute to the severity of a
necrotizing bronchopneumonia.
Primary necrotizing bacterial
pneumonias due to S. aureus, K.
pneumoniae, and P. aeruginosa may
result in bronchiectasis. Streptococcus
pneumoniae, H. influenzae, and
Moraxella infections typically do not cause
bronchiectasis, but may be colonizers
of bronchiectatic airway. Necrotizing
anaerobic pneumonias secondary to
aspiration or bronchial obstruction
are often complicated by parenchymal
destruction and bronchiectasis.
Tuberculosis can result in bronchiectasis
by several mechanisms like as a
consequence of tuberculosis bronchitis,
post obstructive bronchial damage
secondary to post-tuberculosis bronchial
wall stenosis, and extraluminal bronchial
obstruction by enlarged tuberculosis
lymph nodes.
The association of Mycobacterium
avium complex (MAC) with bronchiectatic
airways is well documented.
Bronchial Obstruction
Bronchial obstruction may result in the
development of localized bronchiectasis.
It has been suggested that following
bronchial obstruction, airways proximal
to the collapse are exposed to strong
dilating forces caused by the difference in
the atmospheric pressure in the bronchi
and the negative pressure in the pleural
space. Over time, these forces acting on
weakened, inflamed airways may result
in permanent and pathological airway
dilatation. The presence of surrounding
lung fibrosis, atelectasis, and loss of lung
volume leading to regional increases in
local retractile lung forces may also play
a role.
Bronchial obstruction may facilitate
the development of bronchiectasis by
interfering with bronchial clearance and
promoting bacterial infection, bronchial
wall inflammation and weakening.
Immune dysfunction
Immunodeficiency syndromes such as
immunoglobulin deficiency, complement
deficiency and chronic granulomatous
disease, are associated with bronchiectasis.
Continued on page 3
>>Continued from page 2 Types and pathophysiology
Deficiency of IgG, IgM and IgA put the
patient at increased risk of recurrent
pulmonary infections, which eventually
end in bronchiectasis.
Cystic fibrosis
Cystic fibrosis is well known to
cause bronchiectasis, as a result of
recurrent respiratory tract infections
with Staphylococcus aureus and mucoid
pseudomonas aeruginosa. In addition,
the gene responsible for cystic fibrosis
(CF), the cystic fibrosis transmembrane
regulator (CFTR), is shown to occur in
high frequency in children with idiopathic
bronchiectasis. Clues suggesting CF as a
cause of bronchiectasis include upper lobe
radiographic involvement and sputum
cultures showing mucoid P. aeruginosa.
Young’s Syndrome
Young’s syndrome consists of a
combination of obstructive azoospermia
(with normal spermatogenesis) and chronic
sinopulmonary infections (bronchiectasis
and sinusitis). Young’s syndrome is
distinguished from CF by its lack of family
history, absence of CF genetic mutations,
the presence of normal sweat electrolytes,
and normal pancreatic secretion.
Primary Ciliary Dyskinesia
Primary ciliary dyskinesia (PCD),
phenotypically and genetically
heterogeneous group of conditions with
autosomal-recessive inheritance pattern,
is caused by defects of respiratory cilia,
sperm tails, and cilia of the embryonic
node. Immotile cilia syndrome can lead
to bronchiectasis, as a result of recurrent
pulmonary infections due to retained
secretions. Approximately, 50% of
patients with immotile cilia syndrome
have Kartagener’s syndrome. It consists of
sinusitis, bronchiectasis and situs inversus.
Allergic Bronchopulmonary Aspergillosis
Allergic bronchopulmonary
aspergillosis (ABPA) is a hypersensitivity
Outer wall
Cilia
Mucus
Mucous
gland
Normal bronchus
lung disease caused by the ubiquitous fungus
Aspergillusfumigatus and usually occurs
as a complication of persistent asthma
or cystic fibrosis. The excessive mucus
production and impaired mucociliary
clearance in these conditions manifests as
recurring episodes of asthma, pulmonary
infiltrates, and central bronchiectasis that
may progress to fibrosis.
Inflammatory bowel disease
Pulmonary involvement in
inflammatory bowel disease is uncommon.
Among this majority of cases reported,
have airway disease with bronchiectasis,
occurring in 25% of cases with pulmonary
involvement. Interestingly, some patients
with inflammatory bowel disease develop
bronchiectasis after colectomy. It has
been suggested, that bronchiectasis in
inflammatory bowel disease, is due to an
autoimmune process and infection has a
minor role in its pathogenesis.
Rheumatoid arthritis
The association between rheumatoid
arthritis and bronchiectasis, has
recently received considerable interest.
Bronchiectasis can occur, before or
after the onset of Rheumatoid arthritis.
If bronchiectasis occurs before the
onset of Rheumatoid arthritis, that
chronic suppurative infection leads to
triggering an immune response to the
synovial membrane, causing rheumatoid
a
arthritis. In contrast, those patients who
develop bronchiectasis after the onset of
rheumatoid arthritis may have increased
susceptibility to respiratory infections
caused by rheumatoid arthritis itself or
its treatment. The recurrent pulmonary
infections eventually lead to airway damage
and bronchiectasis.
PATHOGENESIS
In spite of the numerous conditions
that are associated with bronchiectasis,
the underlying cause may be very difficult
to identify. Idiopathic bronchiectasis
represents about half of the case.
Loss of
cilia
Increase
mucus
Destruction
of wall
Bronchiectasis
The basic pathophysiology among all
the conditions that lead to bronchiectasis,
is that they either lead to alteration in the
pulmonary defense mechanisms, or are
associated with inflammation.
Regardless of the initiating event, the
ultimate results in loss of the mucociliary
transport, rendering increased
susceptibility to microbial colonization
that leads to inflammatory response and
progressive lung damage. In the majority,
bacterial colonization and presence of
markers of inflammation in the sputum
are intermittent. The sputum of these
patients contains large amounts of serum
proteins that results from the increased
capillary permeability and exudation of
serum proteins into the alveolar space.
Neutrophils are thought to play a central
role in the pathogenesis of tissue damage
that occurs in bronchiectasis.
It has been found that the sputum
of patients with bronchiectasis has
high levels of neutrophil products,
such as elastase and superoxide
radicals. Neutrophil elastase is a serine
proteinase that has been implicated
in the pathogenesis of bronchiectasis,
emphysema and adult respiratory distress
syndrome. In addition to promoting
tissue damage, Neutrophil elastase also
promotes bacterial colonization mediated
through its destructive effect on IgA, thus
allowing bacterial adherence to the lung
epithelium and affecting the phagocytic
and the complement-fixing activity of
IgG.
A number of inflammatory mediators
like Interleukin 8 (IL-8), Interleukin
Iβ (IL-1β), Interleukin 10 (IL-10),
Interleukin 6 (IL-6), Tumour necrosis
factor a (TNF-a) and Leukotriene β4
(LT- β4) are involved in the recruitment
and activation of neutrophils, in patients
with bronchiectasis. TNF-a leads to the
expression of chemo-attractants and IL-8
leads to neutrophil degranulation.
Endogenous nitric oxide production
is involved in the pathogenesis of
bronchiectasis by direct cytotoxic
effects on the bronchial epithelium and
also through the formation of a highly
cytotoxic compound, superoxide anion.
The current diagnostic test for
bronchiectasis is a high resolution CT scan
of the thorax. Cystic bronchiectasis can
be seen as sac filled structures as routine
Chest X-ray, while ABAP presents with
characteristic radiological features that
can also be seen as a plain Chest X-ray.
Bronchography was a procedure that was
performed until the late 1980s and early
90s, but later became obsolete. This was an
invasive procedure where the dye would
be introduced into the airways through
a rubber catheter introduced in the lung
after locally anesthetizing the vocal cords
and pharynx.
|Volume V, Issue V, November - December 2015| 3
 Dr. Vimal Raj IMAGING OF BRONCHIECTASIS
MD, FRCR, CCT (UK)
Consultant Radiologists
Narayana Health City, Bangalore
Bronchiectasis continues to be a major
cause for repeated chest infections and
physician consultation. Imaging plays
a major role in diagnosis, management
and follow up of patients with suspected
or known bronchiectasis. All imaging
modalities are useful in managing such
patients but each modality has its own
advantage and disadvantage. Therefore, it is
important to choose the correct modality in
a specific clinical scenario. In simple terms,
bronchiectasis is dilatation of the bronchi
in comparison to the adjoining pulmonary
artery. This could either be a primary
phenomenon or secondary to previous
lung injury/adjoining fibrosis (traction
bronchiectasis).
This write up will highlight imaging
appearances of bronchiectasis and
summarise the role of different imaging
modalities.
Chest radiograph (CXR) remains the
most commonly used imaging modality
for patients with bronchiectasis. This is
primarily due to its easy availability, cost
effectiveness, low radiation burden and
relatively comfortable interpretation by
referring physician. CXR may demonstrate
the following findings (Figure 1):
Fig 1: CXR appearances of bronchiectasis. A-
dilated and non-tapering bronchi (Arrow),
B- cystic dilatation of bronchi and C- nodular
opacities in the lungs with dilatation of bronchi
in keeping with mucus plugging.
1. Dilated and/or thickened bronchi
giving a tram track appearance (seen in
longitudinally oriented bronchi)
2. Cystic spaces (typically a cluster of
superimposed cysts), which may or may not
have air fluid levels (suggesting dependent
secretions or active infection).
3. Clustered nodular opacities secondary
to mucus plugging of dilated bronchi (often
seen in Cystic Fibrosis).
4. Consolidation in areas of
bronchiectasis.
5. In Kartagener’s syndrome above
changes are seen along with dextrocardia
(Figure 2).
Computed tomography (CT) is without
doubt the imaging modality of choice
4 RespiMirror|Volume V, Issue V, November - December 2015|
Fig 2: CXR showing dextrocardia with right
aortic arch and dilated bronchi with bronchial
wall thickening (arrows) in keeping with
Kartagener’s Syndrome.
for establishing the diagnosis. It has high
spatial resolution and is not impacted
by summation shadows like CXR. Use
of intravenous contrast is mostly not
required for assessment of bronchiectasis
and is reserved for cases presenting with
haemoptysis to assess for bronchial artery
haemorrhage. Low dose High Resolution
CT (HRCT) is also very useful in diagnosis
and follow up these patients. It is essential
to get good inspiratory scans without
respiratory or movement artefact.
Bronchiectasis are divided into 3 main
types (Figure 3):
Fig 3: Types of bronchiectasis on CT. A-
cystic dilatation of bronchi in keeping with
cystic bronchiectasis, B- Elongated non
tapering bronchi in keeping with cylindrical
bronchiectasis, C- Varicose bronchiectasis with
areas of dilatation alternating with normal
calibre bronchi, especially in the left upper lobe.
◆ Tubular (smooth cylindrical dilatation
of the bronchi)
◆ Cystic type (large dilated cystic spaces)
◆ Varicose (intermittent bronchial
dilatation with intervening normal
bronchial diameter)
On CT the following findings are used
to make a diagnosis of bronchiectasis:
1. Bronchus to adjoining artery ratio of
more than 1 (bronchial enlargement results
in signet ring sign) (Figure 4)
Dr. Tobias Richard A
MD, FRCR, MMed
Consultant Radiologists
Narayana Health City, Bangalore
Fig 4: CT bronchiectasis with dilatation of
bronchi (arrow) in relation to the pulmonary
artery (dotted line) giving the signet ring
appearance.
2. Persistent dilatation of bronchi
without tapering.
3. Visualization of bronchi in the
periphery of the lungs, i.e. within <1 cm of
pleural surface.
4. Bronchial wall thickening of more
than 1 mm. (Figure 5)
Fig 5: Ancillary features of bronchiectasis on
CT. A- cylindrical bronchial dilatation with
surrounding ground glass change and some
fibrosis in keeping with traction bronchiectasis
in a patient with NSIP. B- alternating areas of
air trapping (darker regions) with normal lung
(bright in this image) in keeping with mosaic
attenuation. C- bronchial wall thickening in
dilated bronchi with signet ring appearance.
5. Ancillary findings of endobronchial
mucous plugging, fluid levels within the
dilated bronchi and adjoining consolidation
may also be seen. (Figure 6)
6. Mosaic attenuation (alternating areas
of black and white lung) are often seen
in areas of bronchiectasis secondary to
associated air trapping. (Figure 5)
The distribution of bronchiectasis in
the lungs can provide clues of its aetiology-
Allergic Bronchpulmonary Aspergillosis
(ABPA) associated bronchiectasis is usually
central and upper lobar in distribution,
Hypogammaglobulinaemia related
bronchiectasis is seen in mid zone with
significant bronchial wall thickening and
Kartagener syndrome shows bronchiectasis
Continued on page 8
Allergic bronchopulmonary aspergillosis :
a diagnosis frequently overlooked
Aspergillus, a ubiquitous fungus, affects
the respiratory tract in many ways. The spores
are dispersed by wind in the atmosphere and
it’s the inhalation of these that leads to almost
all forms of aspergillosis. The spectrum of
Aspergillus-associated respiratory disorders
ranges from mild asthma to fatal invasive
disorder and can be categorized into three
well defined clinical categories: allergic
manifestations, saprophytic colonization
of the respiratory tract and invasive
disseminated disease (Table 1).
Table 1. Spectrum of Aspergillus-
associated respiratory disorders
Allergic aspergillosis
1. Allergic bronchopulmonary aspergillosis
(ABPA)
2. Allergic Aspergillus sinusitis (AAS)
3. Hypersensitvity pneumonitis
4. IgE mediated asthma
Saprophytic colonisation
Aspergilloma
Invasive disease
1. Invasive disseminated aspergillosis
2. Chronic necrotising pneumonia
Allergic bronchopulmonary aspergillosis
(ABPA), the most frequently recognized
manifestation of allergic aspergillosis, is an
indolent disease with a protracted course
and occurs worldwide. This clinical entity
was first recognized in 1952 by Hinson
and colleagues from United Kingdom and
subsequently reported from the United States
in 1968. Since then, it has been reported
from all continents and is now seen as an
important emerging disease in India. ABPA,
an immunologically mediated lung disease,
predominantly affects patients with asthma
and also occurs in cystic fibrosis. It is caused by
hypersensitivity to the antigen of the fungus
Aspergillus, especially A. fumigatus (Af).
Repeated inhalation of Aspergillus spores,
principally Af leads to airway colonisation
in susceptible hosts, which evokes an allergic
response, most commonly a type I (IgE
mediated) allergic response. However, type
III (IgG-mediated immune complex) and
type IV (cell mediated) responses too have
been documented.
Epidemiology:
ABPA, as a clinical entity that
predominantly affects asthmatics, was
recognised more than six decades ago.
However, the reason why only a few
asthmatics actually suffer from this,
potentially destructive lung disease is yet to
be ascertained. The exact prevalence of this
disease is not known and it is most likely due
to a lack of uniform diagnostic criterion and
standardised tests. In the mid 1950’s and 60’s
when the awareness regarding ABPA was
restricted only to Europe, it was estimated
that the prevalence of definite ABPA among
asthmatics in England was 8-11% while that
of probable ABPA was 22%. Recent data
suggests that ABPA may be found in up to
6% of all asthmatic patients while in CF, its
prevalence ranges from 2-15%. Denning
and co-workers conducted a scoping review
in order to ascertain the global burden of
ABPA. Data from this study suggested that
the prevalence of ABPA in adult asthmatics is
2.5% (range 0.72–3.5%). A working group on
ABPA complicating asthma was established
by the International Society for Human and
Animal Mycology (ISHAM). This group
compiled the published data on Aspergillus
sensitisation and ABPA since 2000 and
found that the prevalence of Aspergillus
sensitisation among asthmatics varied
between 5.5% and 38.5%. During the same
period, the prevalence of ABPA in patients
with asthma ranged from 2.5% to 22.3% with
a pooled prevalence of 8.4%.
ABPA was first reported from India in
1971. This was followed by the first major
case series from India by Khan and colleagues
from the Vallabhbhai Patel Chest Institute
wherein they presented 46 cases of ABPA
with emphasis on the laboratory aspects.
Chakrabati et al. evaluated 651 patients
with clinically suspected ABPA during an
8-year period of which 89 subjects fulfilled
TABLE 2: Diagnostic criteria for allergic bronchopulmonary asperillosis (ABPA)
Rosenberg–Patterson criteria
Major criteria:
1. Asthma
2. Presence of transient pulmonary infiltrates (fleeting
shadows)
3. Immediate cutaneous reactivity to Aspergillus fumigatus
4. Elevated total serum IgE
5. Precipitating antibodies against A. fumigatus
6. Peripheral blood eosinophilia
7. Elevated serum IgE and IgG to A. fumigatus
8. Central/proximal bronchiectasis with normal tapering
of distal bronchi
Dr Ashok Shah
Director Professor
Department of Pulmonary Medicine
Vallabhbhai Patel Chest Institute
University of Delhi
the diagnostic criteria. In our study of 105
patients with bronchial asthma, ABPA was
diagnosed in eight (7.6%). In a study from
Chandigarh in 564 patients with asthma,
Aspergillus sensitisation was observed in
39.5% and ABPA was documented in 27%.
In spite a high prevalence of ABPA are seen
in different studies from India, ABPA is still
under recognised and under diagnosed.
The striking radiological similarity between
ABPA and pulmonary tuberculosis (PTB)
combined with the high prevalence of
tuberculosis in our country, ABPA is often
misdiagnosed as PTB and these patients
receives anti-TB drugs for years before a
correct diagnosis is made. There is a rampant
misuse of corticosteroids in asthmatics
in our country and this can also mask
the presentation of ABPA. Screening for
Aspergillus sensitisation in patients with
asthma is hardly ever done due to lack of
awareness of ABPA.
Diagnosis:
There has been a gradual evolution of
the diagnostic criterion for ABPA over time.
A set of criteria is required as, apart from
demonstration of central bronchiectasis with
normal tapering bronchi, there is no single test
that establishes the diagnosis or is unaffected
by therapy with oral corticosteroids. The
Rosenberg-Patterson criteria (Table 2) which
is most widely accepted, consists of both
major as well minor criteria for establishing
a diagnosis of ABPA. Central bronchiectasis
with normal tapering bronchi, first
described by Scadding, is considered to be
a pathognomonic feature of ABPA. A set
of minimal essential criteria has also been
advocated by Greenberger, which includes:
1) asthma, 2) immediate cutaneous reactivity
Minor citeria:
1. Expectoration of golden
brownish sputum plugs
2. Positive sputum culture
for Aspergillus species
3. Late (Arthus type) skin
reactivity to A.
fumigatus
Continued on page 6
|Volume V, Issue V, November - December 2015| 5
>>Continued from page 5 Allergic bronchopulmonary
to A.fumigatus, 3) total serum IgE>1000 ng/
mL (417 kU/L), 4) elevated specific IgE-/
IgG-A. fumigatus and 5) central bronchiectasis
in the absence of distal bronchiectasis. The
cut-off values of total IgE and eosinophil
count, as well as the specificity of IgG-A.
fumigatus, for ABPA are not known. In
addition, there is a lack of any consensus
on the minimum number of criteria, either
major or minor, required to confirm the
presence of ABPA. In order to overcome these
pitfalls, a revised criteria for the diagnosis
of ABPA has been proposed by the ISHAM
(International Society for Human and Animal
Mycology) working group wherein the items
are broadly divided into ‘‘obligatory’’ and
‘‘other’’ criteria. This newly proposed set of
criteria (Table 3) recognises bronchial asthma
and CF as predisposing conditions for ABPA.
Table 3: Proposed diagnostic criteria for
ABPA: ISHAM working group
Predisposing Bronchial asthma
conditions Cystic fibrosis
Obligatory criteria(both Type I Aspergillus
should be present) skin test positive
(immediate cutaneous
hypersensitivity to
Aspergillus antigen) or
elevated IgE levels against
A. fumigatus
Elevated total IgE levels
(>1000 IU.mL-1)#
Other criteria (at least Presence of precipitating
two of three) or IgG antibodies against
A. fumigatus in serum
Radiographic pulmonary
opacities consistent with
ABPA
Total eosinophil count
>500 cells.mL-1 in steroid
naive patients (may be
historical)
ISHAM: International Society for Human and Animal
Mycology.
#if the patient meets all other criteria an IgE value
<1000 IU.mL-1 may be acceptable.
The two features of the “obligatory” criteria
are: 1) positive immediate (type I) cutaneous
hypersensitivity to Aspergillus antigen or
elevated IgE levels against A. fumigatus, and
2) elevated total IgE levels>1000 IU/mL. It
is essential that both these findings should
be present for a diagnosis of ABPA. At least
two out of three “other” criteria should be
fulfilled: 1) the presence of precipitating or
IgG antibodies against A. fumigatus in serum,
2) radiographic pulmonary opacities
consistent with ABPA, and 3) a total eosinophil
count > 500 cells/mL in steroid naive patients.
This set of criteria is aimed to help clinicians
establish an early diagnosis. However, this
newly proposed criteria needs ‘‘validation and
further refinement’’.
Though asthma is one of the major
diagnostic criterions, ABPA has been
documented in patients without clinical
asthma. On the basis of their clinical and
6 RespiMirror|Volume V, Issue V, November - December 2015|
radiological findings, more than half of
these patients were initially investigated for
bronchogenic carcinoma while one patient was
referred for evaluation of multidrug-resistant
tuberculosis. This could be attributed to the
absence of clinical asthma and the remarkable
radiological similarities to pulmonary
tuberculosis. It is now thought that a subset
of patients with ABPA present either at an
earlier stage of the disease or with a milder
form. In these patients, central bronchiectasis
is not present but other diagnostic criteria are
fulfilled. Such serologically positive patient
who meet all other ABPA criteria except central
bronchiectasis, are categorised as serological
ABPA and treatment can be initiated to avoid
further chronic lung damage. Although ABPA
has received international attention, it is still
not diagnosed as frequently and as early as it
should be. This results in patients receiving
inappropriate therapy leading to lung damage
that could otherwise have been prevented
Clinical features
Symptoms in ABPA can range from mild
airways obstruction to that of fatal destructive
lung disease. Apart from asthma, ABPA
may also be associated with other clinical
atopic conditions. ABPA, though seen more
frequently in the 20-40-year age group, has
also been reported in children and infants.
Episodes of asthmatic exacerbations with
periods of remissions characterises the
disease process. This ultimately culminates
in a fibrotic lung disease resembling the
chronic fibrocavitary disease of pulmonary
tuberculosis. The frequent expectoration of
golden-brown plugs in the sputum along
with peripheral eosinophilia in poorly
controlled asthmatics should lead to a
strong suspicion of ABPA. The symptomatic
presentation however, appears to bear little or
no relationship to the severity or chronicity
of the disease, as a third of the patients may
be relatively asymptomatic despite extensive
radiological lesions. In our analysis of 113
patients with ABPA, 70 of whom were males,
the mean age of the patients was 32 years
while the mean age of asthma onset was 21
years. Cough (99%) and breathlessness (99%)
were the most common presenting symptoms,
followed by expectoration (98%), wheezing
(97%) and haemoptysis (41%). Fever was seen
in 80% and nasal symptoms in 45%. Sputum
plugs were expectorated by 37% and nasal
plugs by 6%. A personal/family history of
atopic diseases was present in approximately
half of the patients.
Imaging:
Imaging not only helps in establishing the
diagnosis of ABPA, but also helps to monitor
the progress of the disease.
Plain Chest Radiograph
On a plain chest radiograph, a wide
spectrum of roentgenographic appearances
can be seen (Table 4). The changes are
either transient or permanent. Hinson and
colleagues, in their seminal description of
ABPA in 1952, stated, “serial radiographs are
essential to show the sequence of incidents of
Table 4: ABPA: Spectrum of radiological
appearances on chest radiograph
Transient Changes Dilated central bronchi with/
without air-fluid levels
Perihilar infiltrates
Consolidation – unilateral
(Figure 1) or bilateral
Collapse – lobar or segmental
‘Toothpaste’ shadows due to
mucoid impaction in damaged
bronchi
‘Gloved finger’ shadows from
distally occluded bronchi filled
with secretions
‘Tramline’ shadows
representing oedema of the
bronchial walls
Permanent Changes Central bronchiectasis with
normal peripheral bronchi
Parallel-line shadows
representing bronchial
widening
Ring-shadows 1-2 cm in
diameter representing dilated
bronchi en face
Late changes –pulmonary
fibrosis, cavitation, contracted
upper lobes and localised
emphysema
lobar or segmental collapse and consolidation,
first in one part, then in another and in either
lung.” This has been the classical description
of ‘fleeting shadows’, which Hinson et al
recognised as a characteristic feature of ABPA.
These opacities, also known as ‘transient
pulmonary infiltrates’, usually appear and
disappear in different, and sometimes the
same sites in the lung over a period of time,
and affect a segment, a lobe or the whole
lung. These changes reflect disease activity,
and are usually seen in either the acute or the
exacerbation stage of the disease. This is due to
mucoid impaction caused by secretions in the
damaged bronchi and parenchymal infiltrates,
and may clear with or without treatment.
Although these radiological appearances can
closely resemble those seen in tuberculosis
(Figure 1), serial radiographs in ABPA may
reveal the transient nature of these migratory
infiltrates. The upper lobes are predominantly
Figure 1: Chest radiograph showing consolida-
tion in right mid-zone
Continued on page 7
>>Continued from page 6 Allergic bronchopulmonary
involved in ABPA. When these opacities keep
recurring at the same sites they are described
as recurrent fixed shadows. In our review of
1340 chest roentgenograms in 113 patients
with ABPA, we had observed fleeting shadows
in 89 percent. The other transient opacities
included consolidation (91%), perihilar
infiltrates (77%), band (‘toothpaste’) shadows
(65%), ‘V-Y’ shaped (‘wine glass’) shadows
(27%), ‘gloved finger’ opacities (23%), lobar/
segmental collapse (17%), and air-fluid levels
(6%). True hilar adenopathy, which resolved
on therapy, was seen in an adult as well as a
3.5-year-old child.
Permanent opacities reflect irreversible,
fibrotic changes in the bronchial walls and
parenchyma. Unlike transient changes,
these tend to persist throughout life even
when the patient is in remission.The most
pathognomonic radiological feature is the
occurrence of central bronchiectasis with
normal peripheral bronchi, and is considered
as the sine qua non for the diagnosis of ABPA
in patients without CF. The other permanent
changes include parenchymal fibrosis,
cavitation, contracted upper lobes, and
localised emphysema. Parenchymal fibrosis
may present as linear scars, reticulonodular
markings or sometimes as a honeycomb
appearance. Cavitation, though uncommon,
can occur along with the late stages of fibrosis
wherein it may be difficult to distinguish
the disease from fibrocavitary pulmonary
tuberculosis.
Computed tomographic appearances and
demonstration of central bronchiectasis in
ABPA
The spectrum of computed tomographic
appearances in ABPA is highlighted in Table
5. Central bronchiectasis in ABPA has been
considered as the diagnostic of this disease.
Table 5: ABPA: Spectrum of computed
tomographic appearances
Bronchial Bronchiectasis, usually central, as
abnormalities characterized by the ‘‘signet ring’’
and ‘‘string of pearls’’ appearances
(Figure 2)
Dilated bronchi with or without
air–fluid levels
Bronchial wall thickening
High attenuation mucus(HAM)
plugs (Figure 3)
Parenchymal Consolidation
changes Non-homogeneous patchy
opacities
Parenchymal scarring of varying
extent
Segmental or lobar collapse
Cavitation
Pleural Pleural effusion
involvement Pleural fibrosis/ thickening
On plainchest X-ray, central bronchiectasis
appears as parallel line opacities, representing
widening of the bronchi, or as ring
opacities 1-2 cm in diameter, representing
dilated bronchi en face. It is believed that
bronchiectasis occurs in areas with previous
radiological lesions and may result in fibrosis.
Bronchography, once regarded as the “gold
standard” for the demonstration of central
bronchiectasis, gave a one-time complete
picture of the entire tracheobronchial tree.
However, bronchography was considered to
be unsafe in asthmatics.
Currently, computed tomography
(CT) of the thorax has emerged as the
investigation of choice for the demonstration
of bronchiectasis. Our study had shown that
CT, in comparison to bronchography had a
sensitivity of 83% and a specificity of 92% in
detecting central bronchiectasis in patients
with ABPA. Computed tomographic scans
have an added advantage as it has enabled us
to rapidly and safely establish the diagnosis in
children with ABPA who presented with acute
severe asthma. In ABPA, on CT, bronchiectasis
appears as ‘string of pearls’ and the ‘signet
ring’ (Figure 2). In ABPA, bronchiectasis
tends to be predominantly seen in upper
Figure 2: HRCT thorax (lung window) showing
central bronchiectasis as evidenced by
(a) “string of pearls” and (b) “ signet ring” ap-
pearances
lobe in comparison to other pathologies
where a dominant lower lobe involvement
occurs. Bronchiectasis is considered to be
central when it is limited to only the medial
two-thirds or medial half of the lung. High-
attenuation mucus (HAM) plugging (Figure
3), a feature seen on high-resolution CT, has
Figure 3: CT thorax (mediastinal window)
showing high attenuation mucous (HAM) plug
been considered as pathognomonic feature
of ABPA by the ISHAM working group. This
feature was noted in up to 28% of patients
with ABPA. The mucus plug is said to be
hyperattenuating if it is visibly denser than
the paraspinal skeletal muscle. An analysis
of 155 patients with ABPA revealed that
patients with HAM had significantly higher
levels of eosinophils, total IgE and IgE-A.
fumigatus at the time of diagnosis. The other
findings on CT include dilated bronchi
with or without air–fluid levels, totally
occluded bronchi, bronchial wall thickening
and parallel line opacities extending to the
periphery, consolidation, non-homogeneous
patchy opacities, segmental or lobar collapse,
cavitation, fibrosis, contracted upper lobes
and localised emphysema.
Laboratory findings:
Skin testing
Since ABPA is basically an allergic
response to Aspergillus antigens, skin-
prick / intradermal tests have been used to
demonstrate cutaneous hypersensitivity.
Intradermal tests despite give a higher false
positive result. Currently, the skin-prick test
is the favoured technique, serving as a simple
screening tool for ABPA. If the skin-prick
test is negative, then intradermal testing
may be conducted to exclude Aspergillus
sensitisation. Both type I (immediate) and
type III (delayed) responses can be observed
in ABPA. Since skin-prick testing is a highly
sensitive test for ABPA, a negative result in
asthmatic subjects would possibly rule out the
possibility of ABPA.
Eosinophil count
Peripheral blood eosinophilia (>1000
cells/mL) is one of the major diagnostic
criteria and often an initial diagnostic
indicator in a patient with asthma and fleeting
pulmonary infiltrates. However, eosinophilia
is a non-specific finding especially in tropical
countries like India due to a rampant
helminthic infection. In addition, normal
levels may be found in patients already on oral
corticosteroids. Given the poor specificity
of this test, the ISHAM working group has
placed eosinophilia under ‘‘other’’ criteria.
Total serum IgE
Elevated total serum IgE is one of the
minimal essential as well as truly minimal
criteria for diagnosis of ABPA. A patient with
an active disease is unlikely to have a normal
IgE level. Despite it being one of the major
diagnostic criterions, the cut-off level for IgE
remains contentious. The ISHAM working
group has proposed a cut-off level of 1000 IU/
mL, as they ‘‘felt that a cutoff of 500 IU/mL
may lead to over diagnosis of ABPA”.
Specific IgE/IgG to A. fumigatus
Detection of sufficiently high levels of
serum IgE and IgG antibodies specific to
A. fumigatus is also one of the minimal
essential criteria that need to be fulfilled for
the diagnosis of ABPA. As mentioned in the
minimal essential criteria for diagnosis, the
serum values of IgE- and IgG-A. fumigatus in
patients with ABPA should be at least double
the pooled serum samples of patients with AIA.
If values from the controls are not available for
comparison, then, as suggested by the ISHAM
working group, an IgE-A. fumigatus level
>0.35 kUA/L could be considered. However,
Continued on page 16
|Volume V, Issue V, November - December 2015| 7
 HORSE LUNG POWER Mr. Nitin Vanjare
Chest Research Foundation, Pune

The horse is a matchless

athlete with tremendous power and
endurance. It is the only animal of its kind
that can bear the weight of a human and
can still sprint at speeds of 55 kph and
more. The respiratory system of the horse
plays an important role in its spectacular
physical performance.
Like most animals, the respiratory
system of the horse consists of nostrils,
nasal passage, pharynx, larynx, lungs
and the alveoli. However, horses cannot
breathe through their mouth and are
obligate nasal breathers. This is the reason
why horses cannot pant as a method of
Figure 1: a) Breathing through the nose b) During swallowing
thermoregulation as seen in dogs and
humans.The respiratory system starts with Oral breathing can occur only in horses high cardiac output, has more number
the nostrils, which has an outer cartilage with significant anatomical abnormalities of capillaries and mitochondria in the
that keeps the nostrils open during or pathological conditions.The soft palate skeletal muscle (see table 1).
inspiration. The nostrils open into the and the epiglottis are the two flaps of tissue The horse spleen stores around 30-35%
nasal passages. The nasal passages have that prevent food and other material from of the total red blood cells, compared to
conchae (shell like structure) on either entering into the horses’ lungs (figure 1a). around 10% in humans. This functions as
sides which helps in increasing the surface The epiglottis rests above the soft palate a lactate sink. Horses have high muscle
area available for the air. while the animal is not swallowing, forming oxygen reserves which is freely available
an airtight seal. substrate for ATP synthesis. They have
Horse Human (70Kg and 30 While swallowing high muscle buffering capacity which
(400Kg) years old) the epiglottis, helps them to tolerate high lactate
soft palate and concentrations during exercise and delay
1 Lung area Size of Size of one tennis fatigue. Their muscle buffering capacity is
arytenoid cartilage
10 tennis court reposition (figure ~60% more than humans.
courts 1b) and thus the During a sprint, the horse breathes
food enters into in coordination with every stride. As
2 Total Lung Capacity ~ 55 L ~5 L
the stomach. the horse's ab muscles pull the hind legs
3 Oxygen uptake at rest ~ 1600 ml/ ~ 250ml/min Among the various forward in the "suspension" phase of the
min physiological sprint, the organs within the abdominal
factors that cavity are pushed backward, therefore
4 Oxygen uptake during ~ 60 L/min ~ 6 L/min improve the bringing air into the lungs (inhalation).
exercise horses’ athletic As the neck is lowered during the
competence, the extended phase of the sprint, the hind
5 Cardiac Output at rest ~28L/min ~ 5L/min ability to uptake legs move backwards and the "guts"
6 Cardiac Output during exer- ~250-280 L/ ~ 20- 40L/min maximum oxygen of the horse more forward, pushing into
cise min is the major factor, the diaphragm and forcing air out of the
which is more lungs (exhalation). The resistance for
7 Heart rate at rest ~26 – 42 / ~ 60-100 /min than twice that the passage of air in the human lungs is
min of an elite human amongst the lowest in all adult mammals.
athlete. Moreover, The resistance for the passage of air in the
8 Heart rate during exercise ~200-240 / ~150-190 /min
the horse has human lungs is amongst the lowest in all
min an extremely adult mammals.

>>Continued from page 4 IMAGING OF BRONCHIECTASIS

in middle lobe and lingula.
Traction bronchiectasis is seen
in areas of fibrosis. (Figure 5)
CT scoring systems have also
been developed to objectively
quantify the disease and for
follow up. Low dose HRCT
is useful for follow-up and
in surveillance of patients
with cystic fibrosis. Magnetic
Resonance Imaging (MRI)
Fig 6: Complications with bronchiectasis such as fluid levels (A) has limited role in imaging
and nodular infective changes (arrow in B) and consolidation of bronchiectasis due to poor
(broken arrow in B). signal from air within the
lungs. It has an established role
in functional assessment of
lungs to assess the ventilation
and perfusion. Many centres
have also started using MRI in
surveillance of patients with
cystic fibrosis to limit radiation
exposure to young patients.
In summary, CT is the
modality of choice in establishing
the diagnosis of bronchiectasis.
Follow up of patients and during
acute exacerbation CXR is
useful.

8 RespiMirror|Volume V, Issue V, November - December 2015|

 PHYSIOTHERAPY IN BRONCHIECTASIS Dr Nikita Jalan
Master of Physiotherapy (MPTh)
Cardiovascular and Respiratory Sciences
Chest Research Foundation, Pune

Patients of Bronchiectasis regularly 8. Oxygen delivery device

present with the symptoms of persistent 9. Stethoscope Apical
Apical
and
Posterior
Fused
into
single
productive cough, voluminous quantities 10. Pulse oxymeter Upper Posterior Anterior
element
Upper

of sputum, dyspnoea, exercise intolerance Anterior Superior

Lingular

and avoidance of the physical activity

Lateral
What should you monitor? Middle Medial
Interior

Superior
which inevitably affects their quality of life. 1. Vital signs(Heart Rate, Respiratory Superior
Anterior basal
Anterior Basal Segmental Lower

As the disease severity progress, it starts Rate, Blood Pressure, Oxygen saturation)
Medial basal
Medial Basal
Lower
Lateral Basal
(Teriteray) Lateral basal

affecting lower parts of the airways which 2. Breathing patter(symmetrical rise

further increases the difficulty of clearing
the airways. This leads to the vicious cycle
of repeated respiratory tract infections.
Bronchiectasis is often complicated by
pneumonia, recurrent lower respiratory
tract infections, pulmonary hypertension
causing right ventricular hypertrophy and
heart failure in the long run.
It is important for patients of
Bronchiectasis to perform regular chest
physiotherapy to maintain their lung health
and prevent complications of the disease
process. Pathologically there are damaged
cilia in the lower airways, which increase
the difficulty of airway clearance. There is
associated poor nutrition and poor systemic
hydration. These patients often complain
of general tiredness and weight loss and
this is attributed to increased caloric
expenditure required for coughing and
clearing their secretions. With these clinical
presentations, physiotherapy has been long
regarded as one of the most important
aspects of treatment for bronchiectasis.
of thoracic wall, synchronised thoraco-
abdominal movement, use of accessory
muscles)
3. Subjective response- pain, discomfort
& light headedness
4. Dyspnoea
5. Change in skin colour
a) POSTURAL DRAINAGE (PD):
Here the patient is positioned in
such a way that the affected broncho-
pulmonary segment is directed close to
the main bronchus. The targeted lung
segment is nearly perpendicular to the
ground and gravitational force assists in
passively draining the secretions from these
dependent segments. The time required
is 3-15 minutes for each segment (daily)
depending on patient’s hemodynamic
stability and properties of the secretions.
Postural drainage assists in removal of the
secretions from the peripheral airways
towards the more proximal bronchi near
the hilum. It is essential to have a sound
Posterior basal
Posterior basal Bronchi
FIGURE 2: View of Bronchopulmonary segments
of right and left lung
lumen during PD position. The patient
can perform percussion independently
(areas of accessibility) or with assistance.
A cup is formed of both the hands and it
is rhythmically stroked on the patient’s
thorax for 2-3 minutes on the involved
lung segment, covered by a thin sheet of
clothing. It should be a hollow sound and
not a clapping sound that should be heard
while per cussing a patient. Care should
be given for avoiding breast tissues, bony
prominences and floating ribs. The energy
developed by striking is transmitted through
the thoracic wall into the lungs. There are
various pneumatic devices available which
mimics the function of percussion (e.g.:
hand held mechanical precursor, baby
mask etc)

Chest Physiotherapy(CPT)
This is a technique which helps to
mobilize secretions from the lower airways
into the upper respiratory tract and finally
coughed out. CPT includes postural
drainage, percussion, vibrations and
assisted coughing/huffing techniques which
help to mobilize the secretions towards the
central airways. These techniques can be
used in isolation or together with various
combinations according to the need of
the patients. CPT can be performed along
with administration of bronchodilators
and mucolytics as they help loosen the
secretions.
What equipments are required?
1. A Bed
2. Pillows for supporting patient’s
positions
3. Thin towel for percussion
4. Tissues/basin for collecting sputum
5. Suction equipment for patients who
are unable to clear secretions
6. Gloves, masks, goggles and gowns
as indicated for protecting caregiver from
infected secretions
7. Optional: Hand held mechanical
percussor/vibrator
FIGURE 3: Cup formation FIGURE 4: Percussion
for percussion being applied to the left
anterior Apical Segment
to a patient
FIGURE 5: Baby mask (External percussor)
c) VIBRATIONS:
As with percussion, vibrations involve
loosening and dislodging the secretions
FIGURE 1: PD position for Bronchopulmonary segment
(Image taken form In Wilkins RL. Egan’s Fundamentals of Respiratory from the bronchial walls into the lumen of
Care; ed 9. St. Louis, 2009, Mosby) the airways. Gentle vibrations are provided
knowledge of the lobes and the broncho- with flat hands fixed into each other by
pulmonary segments to achieve optimal voluntarily contracting the shoulder
drainage of secretions. Fig 1 depicts 12 PD muscles. Vibrations are applied once the
position for broncho-pulmonary segments. patient exhales in PD position. They are
Fig 2 depicts the bronchopulmonary generally applied following percussions in
segments of right and left lung. PD positions on the affected lung segments.
It is wise to practice this method on a
b) PERCUSSION: rubber hot water bag before administering
This aids in sputum dislodgement or to a patient. It should be done for not more
loosening from the airway walls into the Continued on page 10

|Volume V, Issue V, November - December 2015| 9

>>Continued from page 9 PHYSIOTHERAPY IN BRONCHIECTASIS
FIGURE 6: Vibration being given to patient in PD position
with flat hands
than 5 -7 minutes. A mechanical vibrator
may be used as an alternative to manual
vibrations.
d) ASSISTED COUGHING and HUFFING
TECHNIQUES:
As these patients generally complain
of weak cough and retained tenacious
secretions, physician can aid them by the
technique of assisted coughing or huffing
Assisted coughing- It is a forced expiratory
technique performed by the patient with
closed glottis. The caregiver/doctor supports
with assisted compression on the chest wall
during coughing. This technique works by
assisting the respiratory muscles towards
forceful expulsion of the tenacious secretions
from the airways. This technique is suitable
for patients with weak cough.
FIGURE 7: The caregiver provides assisted compression to
the thoracic wall during coughing
Steps for assisted coughing:
◆ Caregiver places the heels of her hand
below the rib cage
◆ Patient performs deep inhalation
◆ Holds the breath for 2-3 seconds against
closed glottis
◆ Patient forcefully expels the air out
(sound like- coughhhhh). Caregiver
assists the patient during his ineffective
cough by providing chest compression in
upward and inward direction during the
coughing manoeuvre.
◆ Process is repeated 2-3 times
Huffing- It is a forced expiratory technique
performed by the patient with open glottis
resulting in lower intrathoracic pressure
than a cough. This technique is suitable for
patients who are unable to generate the flow
required to cough.
STEPS for Huffing:
◆ Patient performs deep inhalation
◆ Holds the breath for 2-3 seconds against
open glottis
◆ Forcefully expels the air out keeping
the back of the throat open (sounds like
hufffff, patients should be instructed as if
he is steaming the glasses to clean them)
◆ Process is repeated 2-3 times
Deep breathing and coughing:
When the patient is being provided with
PD, percussion or vibrations, they should
be instructed to take relaxed deep breaths
intermittently followed by active/assisted
coughing or huffing so that mucus can be
easily expectorated as the secretions have
now moved centrally towards the larger
airways.
How to perform CPT?
◆ Auscultate patients breath sound and
identify the broncho-pulmonary segment
requiring CPT
◆ Look for last 2 hours food intake( patient
should not consume a heavy meal 2 hours
prior to CPT)
◆ Assess the requirement for CPT
◆ PD positons (~ 10 minutes)
◆ Percuss and Vibrate the required broncho-
pulmonary segment in PD Position
◆ Active or assisted cough/huff
◆ Re –assess by ausculating the breath
sound
◆ Repeat the procedure for other lung
segments (if required)
When should you not perform CPT?
◆ All recent surgeries (4 weeks)
◆ Acute or unstable Head, Neck and Spine
injuries
◆ Acute Myocardial infarction(4 weeks)
◆ Recent frank haemoptysis during last 4
weeks
◆ Pulmonary embolism and oedema
(untreated)
◆ Unco-operative, anxious, incomprehen-
sive patients
◆ Open wound on the body parts
How will you evaluate the outcome of CPT?
◆ Improvement in chest X-ray after
treatment
◆ Improved breath sound
◆ Improved oxygenation (seen on Arterial
Blood Gas, Pulse oxy meter)
◆ Patients subjective response of feeling
better
◆ Improved sputum expectoration (sputum
quantity)
Respiratory physiotherapy Devices:
These devices are alternatives that are
available for patients to manage their daily
sputum clearance. It reduces the dependence
of patients on caregivers for CPT and also
assists in easy airways clearance.
Physiotherapy adjunct devices have
proven to be equally effective in airway
clearance like the manual CPT. As no single
inhalation device is fit for a patient so is the
Respiratory physiotherapy technique and
Continued on page 11

S. No NAME PRINCIPLE HOW TO USE THE DEVICE REMARKS

1 Positive Expiratory pressure (PEP) PEP therapy works on the
device principle of development of
positive pressure while exhaling
against resistance into the device.
It aids in reversing atelectasis and
clearing mucus by splinting the
airways open and increasing the
intrathoracic pressure distal to the
retained secretions.
Pressure Indicator
Mouthpiec
Resistor dial
1.Patient should sit comfortably and There are various models of
upright while holding the mouthpiece PEP devices available in the
tightly between the lips market (with mouthpieces &
2. Resistor dial is adjusted according to facemasks)
the patient’s ability of exhale forcefully
against the resistance The cost of PEP device ranges
3. Patient takes a deep breath (larger from 1500-2000INR
than normal tidal breath) but not to
total lung capacity. PEP devices have been
4. Followed by exhalation, maintaining proven to be effective in
a prescribed pressure of 10–20 cm airways clearance in patients
H2O on the pressure indicator. with Bornchiectasis.
5. Exhalation time should last ~3 times
longer than inhalation.
6. Patient should perform 10–20 PEP
breaths, followed by 2–3 forced coughs
or huffs.
7. Steps 3–6 are repeated until
secretions are cleared or until 10-15
minutes of treatment time.

10 RespiMirror|Volume V, Issue V, November - December 2015|

>>Continued from page 10 PHYSIOTHERAPY IN BRONCHIECTASIS
S. No NAME PRINCIPLE HOW TO USE THE DEVICE REMARKS
2 Acapella
3 Flutter
Protective Cover
Circular Cone
High density stainless
Mouth piece
4 High Frequency chest
oscillation(HFCWO)
device. Patients should
be able to decide what
is best for them under
the expertise of their
treating physiotherapist.
There are various devices
available as adjuncts to
CPT. Positive Expiratory
pressure (PEP) device,
Acapella, Flutter, High
Frequency chest wall
oscillation etc.
It combines the principles of
PEP therapy and high frequency
oscillations.
When patient exhales into the
device, the magnets placed inside
it oscillates. These oscillations are
transferred to the airways which
aid in dislodgement of the sputum
from the airways
This device also works on the
principles of PEP therapy and high
Perforated frequency oscillations.
Flutter consists of a perforated
steel ball
cover and a circular cone with a
steel ball placed inside it. When
patient exhales into the device,
the steel ball vibrates in the
casing against exhalation. These
vibrations are transferred to the
airways which aid in dislodging
the tenacious sputum from the
airways
It is a mechanical form of CPT.
wall
It consists of an inflatable vest
connected by tubes to pulse
generator.
The vibratory force generated
by HFCWO lowers the mucus
viscosity and assist in easy
expectoration of the sputum.
FIGURE 12 FIGURE 13: FIGURE 14:
Patient is Patient is
seated with arm seated with arm standing with arm
supported on supported
thigh on table
1. Patient should be in any Acapella is available in two
comfortable position (upright models. Green model is
sitting, reclined or PD Position) suited for adults and blue
2. Patient inhales deeper than normal model is suited for children
breath and holds the breath-hold and geriatric population.
for 2-3 seconds.
3. Patient places the mouthpiece Acapella is easily available
of Acapella device in the mouth and the cost of varies from
and exhales at twice the flow of 2500-3000INR
a normal exhalation. Exhalation
is continued until lungs reach
functional residual capacity.
4. Patient should perform 1-2 high
flow expiratory breaths, followed
by 2–3 forced coughs or huffs.
5. Steps 2–4 are repeated until
secretions are cleared or until 10-
15 minutes of treatment time.
1. Patient should sit comfortably and Acapella and Flutter have
upright with relaxed breathing. been proven to be equally
2. Patient inhales deeper than normal efficient in airway clearance
breath and holds the breath-hold and the choice of device is
for 2- 3 seconds. dominated by the patient.
3. Patient places the Flutter device
mouthpiece in the mouth and
exhales at twice the flow of a Flutter is easily available and
normal exhalation. Exhalation the cost of varies from 4000-
is continued until lungs reach 4500 INR
functional residual capacity.
4. During exhalation through the
Flutter, patient should always point
the perforated cover upwards.
5. Patient should perform 1-2 high
flow expiratory breaths, followed
by 2–3 forced coughs or huffs.
6. Steps 2–5 are repeated until
secretions are cleared or until 10-
15 minutes of treatment time.
1. Patient attains a comfortable It is not suitable for patients
position (upright sitting, reclined who are osteoporotic & have
or PD Position) external lines placed on their
2. Vest is worn over a thin layer of torso(chest tube, drains etc)
clothing
(the vest extends from the shoulder HFCWO is a relatively
to the hip bone) expensive device, used mostly
2. HFCWO is switched ON. in hospital settings.
3. The vest rapidly inflates and
deflates, compressing and releasing HFCWO has been proven
the thoracic wall up to 20 times per to be effective in airways
second. clearance in patients with
4. Patient performs coughing / Bornchiectasis
huffing following 10-15 minutes of
HFCWO Approximately the cost varies
from 1,50000- 3,00000INR
FIGURE 15:
Patient is Patient is standing
with back FIGURE 16: Patient is in lying
supported against position with head and limb
supported the wall and arm supported by pillows
on table supported on thigh Continued on page 13
|Volume V, Issue V, November - December 2015| 11
 POST PULMONARY TUBERCULOSIS
BRONCHIECTASIS – THE SIDE PLOT
Introduction
Bronchiectasis is defined as a persistent
or progressive condition characterized
by dilated, thick walled bronchi. Post
pulmonary tuberculosis bronchiectasis
(PTBB) is defined as cylindrical or saccular
dilation of the bronchial tree occurring
in an area of previous tuberculosis.
The association of bronchiectasis with
pulmonary tuberculosis (PTB), especially
post treatment has been known since the
times Laennec and was first described by
Grancher in 1878. However for a long
time since then the fight was to achieve
cure and somehow survive, rather than
to worry about post survival problems.
It is only in the last fifty years that the
availability of effective drugs has increased
the survival of TB patients and thereby
it’s associated sequelae. Even though new
problems like MDR and XDR TB have
reared their heads, overall the fight seems
to be progressing towards control.
The battle scene is shifting towards life
after PTB and its resulting disabilities.
Many studies have shown that the
disability due to PTB is primarily due to
disability after cure has been achieved.
This article deals with one of the post PTB
diseases – bronchiectasis.
Etiopathogenesis
Many studies have reported varying
incidences of Post TB bronchiectasis
(PTBB) depending on the definitions
taken into consideration and on the
investigative and confirmatory methods
used. They are generally in the range of
60% - 100%. However, there has been an
undeniable decrease in the incidence and
severity over time.
PTBB may manifest in three situations:
• Bronchiectasis in a cured case of
tuberculosis.
Figure1: Post TB Bronchiectasis
12 RespiMirror|Volume V, Issue V, November - December 2015|
• Bronchiectasis in an active case of
tuberculosis.
• Tuberculosis superimposing on an
established case of bronchiectasis.
However the investigations, diagnosis,
management and prognosis remain more
or less the same in all three situations and
need not be differentiated as such.
The possible scenarios for the
etiopathological evolution of PTBB could
be development of tuberculous bronchitis
resultant to spillage of tuberculous exudate
from a parenchymal focus or ruptured
lymph nodes into the bronchial tree. This
gives rise to damage to the epithelium, loss
of cilia and mucus glands and weakening
of both elastic and muscular components
of the bronchus along with contraction
of the surrounding connective tissue.
This damage is precipitated by the
action of enzymes like collagenases and
metalloproteinases. Another possible
mechanism is secondary to bronchial
obstruction due to hilar tuberculous
adenopathy compressing adjacent bronchi
with secondary infection. Traction
bronchiectasis may result due to the pulls
and pressures of adjacent diseased fibrotic
tissues. Each of these etiopathological
factors may act in isolation or confluence
leading to the final picture of PTBB.
Various pathological stages and
differentiations of PTBB like: cylindrical,
varicose and saccular or traction, mixed,
saccular, cystic and central have been
proposed by different authors based on
radiological or morphological features.
However, these classifications are grossly
of academic interest only and don’t serve
any significant purpose in the diagnosis,
prognosis or management of these
patients. But the one fact that is for sure is
that the more severe the original disease,
or more imperfect the
treatment taken, the
more is the likelihood
of development and
severity of PTBB.
Also, concomitant
noxious influences
like pollution,
smoking, biomass fuel
exposures contribute
to the aggravation of
symptomatology and
prognosis of these
patients.
The classical
presenting complaints
in patients of PTBB
are no different
Dr Stani Francis Ajay
MD TB & Respiratory Diseases
Professor & HOD
Dept. of Respiratory Medicine
S.B.K.S Medical Institute &
Research Centre Vadodra,Gujarat
from any other case of bronchiectasis –
cough with copious sputum - mucoid,
mucopurulent or purulent. Dyspnea,
hemoptysis, pain and fever may be
present in varying proportions. Infective
exacerbations are marked by worsening
of baseline symptoms and/or appearance
of new symptoms. A wide range (from
3 to 12 episodes per year) of frequency
of exacerbations has been reported in
different studies. The impact of the disease
on the psyche of the patient and his/her
quality of life has also been documented
by other authors.
The chief clinical finding is the presence
of crackles on auscultation during both
phases of respiration, but especially
on expiration. Intensity of crackles
may reduce temporarily after cough.
Localization of these crackles correlates
poorly with actual areas of bronchiectasis.
Other findings like wheeze and finger
clubbing are inconsistently found.
The investigations in case of PTBB
revolve mainly around the exclusion of
active PTB and ruling out other underlying
causes of bronchiectasis. The full battery
would include blood inflammatory
markers, first line and second line
immunological tests, gastrointestinal
investigations, investigations to exclude
cystic fibrosis and tests of ciliary function.
However, pure cases of PTBB would
probably need only a detailed history,
examination and basic radiology to stamp
the diagnosis.
A plain chest radiography and HRCT
of thorax are the only radiological
investigations needed in PTBB. Though
there are problems of sensitivity in chest
radiography, it is probably the commonest
investigation done in these patients for
diagnosis as well as for follow up. HRCT
thorax is considered the gold standard in
diagnosis of bronchiectasis for all practical
purposes. But it has its limitations too,
especially in early cases and in the presence
of confounders like age and smoking.
Two investigations of corroborative
nature are sputum microbiology and lung
function tests. Both these tests have a role
in recording the baseline and monitoring
the exacerbations and reliefs. Sputum
microbiology, done under ideal test
conditions, also guides antibiotic therapy.
The role of bronchoscopy in the
diagnosis and treatment of established
cases of PTBB is limited. At best it may be
used to increase the microbiological yield
Continued on page 13
>>Continued from page 12 POST PULMONARY TUBERCULOSIS
in stubborn cases or in suspected cases of
atypical mycobacterial infection.
The goals of treatment as
outlined in the BTS guidelines
are
• 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.
The main stay of treatment to achieve
these goals is respiratory physiotherapy.
There is a whole armamentarium of
techniques and devices available. These
include active cycle of breathing exercises,
postural drainage and its modifications,
positive expiratory pressure (PEP),
oscillating PEP devices, manual techniques,
autogenic drainage etc. The effective strategy
seems to be to acquaint the patients to all the
possibilities and let them chose according
>>Continued from page 11 PHYSIOTHERAPY IN BRONCHIECTASIS
Dyspnoea relieving positions
Patients of bronchiectasis are generally
breathless considering increased use of
accessory muscles (shoulder muscles),
altered gas exchange at alveolar level
and physical de-conditioning. Dyspnoea
relieving positions work by relaxing the
shoulder muscles which reduces the oxygen
requirement of the accessory muscles leading
to reduced dyspnoea.
Deep Breathing exercises (DBEx)
DBEx improves ventilation and reduces
the work of breathing of accessory muscles.
It also reduces dysponea, respiratory rate and
the occurrence of atelectasis in patients who
are shallow breathers due to their increased
work of breathing. Patients of bronchiectasis
should engage in performing DBEx twice
daily with 3-4 deep breathes.
STEPS
◆ Patients attains a semi-lying position with
head supported
◆ Pillows are placed under the knees
◆ Patients/caregiver places his hand over the
abdominal region (to provide sufficient
to their convenience. This is so because
physiotherapy is most effective when used
consistently and compliance is likely to be
high for a patient driven regimen than for
a physician handed down one. Roughly
20 – 30 minutes of physiotherapy 2 times
a day suffices for majority of the patients.
Airway clearance techniques can be made
more effective by adjunctive measures
like humidification, intermittent positive
pressure breathing, nebulised saline or
hypertonic saline.
The pharmacotherapy of PTBB is
limited to antibiotics and bronchodilators.
There is no evidence to support the routine
use of mucolytics, hyperosmolar agents,
xanthines, inhaled and oral corticosteroids.
Antibiotic use in treatment of patients
of PTBB is broadly guided by culture-
sensitivity reports. All patients with
purulent component in their sputum are
not candidates for antibiotics. There has
to be significant worsening of symptoms
with systemic effects to warrant start
of antibiotics. Usually monotherapy is
sufficient. Dual regimen is needed only
for documented cases of resistant strains.
Long term antibiotics are advisable in
cases having three or more episodes of
exacerbation in a year or lesser number of
episodes with accompanying significant
morbidity. Nebulised antibiotic regimen
have also been proved to be useful in these
patients especially children.
There is a well defined cohort of patients
who would benefit from lung resection
visual input to the patient)
◆ Patient is instructed to take a deeeeeeeep
breath slowly through the nose, feeling
the expansion of the abdomen under the
caregiver’s hand
◆ Patient holds the breath for 2 to 5 seconds
◆ Followed by exhalation through the
mouth
FIGURE 17: Patient is in semi lying position and is
being assisted by caregiver for deep breathing
Exercise and physical activity
Patients generally complain
surgery. Disease requiring localized
resection (ten lung segments left back
seems to be optimum) and which has not
responded adequately to maximum medical
management is an adequate indication for
surgery.
In terms of disease distribution,
bronchiectasis affecting areas of lung not
amenable to natural drainage like, lower
lobes or the lingula may need surgical
intervention. Massive hemoptysis also
constitutes an indication for surgery;
though bronchial artery embolization may
also be tried in these cases. The option of
lung transplantation can also be considered
in very selective cases.
To conclude, a patient of pulmonary
tuberculosis presents with cough,
expectoration, hemoptysis and respiratory
compromise – the same set of complaints
which a patient of PTBB is likely to have.
While it is all so heroic for physicians to
have diagnosed the patient, treated it fully,
achieved microbiological cure and then
gone on to diagnose PTBB, as far as the
patient is concerned, s/he is still where s/
he started. Thus post treatment problems
like PTBB pose special challenges to all
stakeholders involved – the patient, the
physician and the policy maker.
These challenges need to be recognized,
highlighted and appropriately addressed in
order to win this battle against one of the
oldest microbiological enemies of mankind
in a more comprehensive and meaningful
manner.
exercise intolerance as a consequence of
de-conditioning of the skeletal muscles.
In order to achieve greater exercise fitness
levels, patients should engage in daily
walking/cycling/swimming for 20-30
minutes.
To accomplish tasks of daily living, an
individual requires sufficient upper and lower
limb muscle strength and co-ordination
which can be achieved through exercises
that are specific for extremities. Thus,
patients should perform strength training for
their extremities using free weights on two
alternate days a week. Daily engagement in
exercise also facilitates airway clearance in
these patients due to improved ventilation
which leads to enhanced dislodgment of the
mucus from the distal airways.
Physicians should periodically re-assess
patients at intervals of 3 months for airway
clearance, exercise adherence and general
wellbeing after participating in physiotherapy.
Effective treatment is recognised by
reduced daily sputum, improvements in
spirometry, improved oxygen saturation,
reduction in breathlessness along with
improved ability to carry out day to day
of activities.
|Volume V, Issue V, November - December 2015| 13
 Cystic Fibrosis in India:
Are We Missing Diagnosis
SK Kabra
MBBS, MD (DNB)
Dept of Pediatrics AIIMS, New Delhi

Cystic fibrosis (CF) is an autosomal manifestations are highly variable. Clinical
recessively inherited life limiting illness, manifestations in Indian children with CF
first described by Dorothy Anderson in have been reported to be similar to that of
1938. It was considered to be disease of Caucasian population.
Caucasian population. More recently it The frequency and severity of the
is recognized that CF occurs in all ethnic symptoms is variable due to delayed
groups with frequency varying from diagnosis in Indian children. Available
1:2500 in United Kingdom to 1 in 80000 in information suggests that the age of
Native American population. diagnosis of CF in India is between 3-5
Cystic fibrosis was considered to be years, compared to 6 months in Caucasians.
non-existent in India, until 1968 when The clinical manifestations observed in a
the first case was reported. Over the past large series of 120 children from All India
few decades there are increasing reports Institute of Medical sciences, New Delhi are
of CF from almost all parts of India. The given in Table 1. Common manifestations
precise magnitude of the problem of CF include recurrent/ persistent pneumonia
in India is not known as there are no and malabsorption with failure to thrive.
community based studies on the subject. The disease was found to be more severe at
Some estimates based on incidence of the time of diagnosis as indicated by lower
CF in migrant population in USA and CF clinical scores (Schwachman’s score).
UK suggests it to be between 1:10000 to Other manifestations that are different
1: 40000. An estimation based on study from case series reported from developed
of carrier state of ΔF508 mutation on countries include, hypochloremic
950 cord blood samples suggests it to be
Table 1. Clinical characteristics of children with cystic fibrosis
1:40000 newborns.
The total estimated live birth in India Characteristics Frequency (N = 120)
in the year 2012 was 27, 271,000. Based Demography
on this, the number of newborn with CF Mean age at diagnosis 54 months (95% CI 3–154)
born annually may be 10908, 2727 or Mean age of onset of symptoms 11 months (95% CI 0.1–60)
681 with a presumed incidence of 1:2500, Boys 80 (67%)
1:10000 or 1:40000 respectively. Majority
Girls 40 (33%)
of these infants may not survive as their
Symptoms
diagnosis is very often missed and they do
not get appropriate treatment. The cause of Persistent/ recurrent pneumonia 118 (98%)
death in them is attributed to commonly Failure to thrive 108 (90%)
prevalent conditions like malnutrition/ Malabsorption 96 (80%)
pneumonia or diarrhea. Rectal prolapse 16 (13%)
The basic defect in cystic fibrosis is a Dehydration 16 (13%)
defective cystic fibrosis transmembrane Meconium ileus 10 (9%)
conductance regulator (CFTR) gene. The
Vitamin A deficiency 10 (9%)
gene was identified in 1989 and is located
on the long arm of chromosome number Salt craving 5 (5%)
7 at position 7q31.2. Currently, there are Salty taste 5 (5%)
about 2000 mutations listed in the CFTR Skin rashes 5 (5%)
mutation database. The mutation profile Vitamin D deficiency 4 (4%)
is well documented in the Caucasian Pneumothorax/empyema 3 (3%)
population and in them the commonest
Meconium ileus equivalent 2 (2%)
mutation is ΔF508, which constitutes more
Signs
than 70%. However, the mutation profile
Normal or mild malnutrition 70 (58%)
of Indian children with CF is not very well
characterized. Various reports suggest that Severe malnutrition 50 (42%)
ΔF508 mutation is relatively less common Z Scores for weight for age - 2.59 (95% CI: - 3.01 to
-2.32)
in children from Asia. Review of all the
reports from India suggests frequency of Clubbing 80 (75%)
ΔF508 mutation to be between 19 to 56%. Chest
It is also observed that the mutation profile
Crepitations 110 (92%)
is very heterozygous in India, possibly due
Hyperinflation 100 (83%)
to variation in ethnic backgrounds.
Clinical manifestations of cystic fibrosis Rhonchi 40 (33%)
Cystic fibrosis is a multisystem Bronchial breathing 20 (17%)
disorder predominantly affecting lungs Nasal polyposis 5 (4%)
and the gastro-intestinal system. Clinical CF score mean (95% CI) 51 (20–80)
metabolic alkalosis, vitamin A and D
deficiencies, higher colonization rate with
Pseudomonas and lower rates of common
mutations.
Diagnosis
Diagnosis of CF is based on
demonstration of CFTR dysfunction in
presence of clinical phenotype (Table 2).
Table 2. Diagnostic criteria for cystic fibrosis
One or more characteristic clinical features
- Or a positive newborn screening test result
- Or a history of CF in a sibling
AND
Increased sweat chloride concentration by
pilocarpine iontophoresis method on two or
more occasions
- Or identification of two CF causing
mutations
- Or demonstration of abnormal nasal
epithelial ion transport
Due to non availability of panel of
common mutations and equipments
for demonstration of
abnormal transepithelial
potential difference, high
sweat chloride values
remains the gold standard
for diagnosis of CF in
India.
At present sweat testing
facilities are not available
widely in India and that
is a major cause of under
diagnosis of CF. Even if it is
suspected by pediatricians,
they are not able to
confirm the diagnosis. An
inexpensive and simple
indigenously developed
sweat testing method
has been developed and
validated for use in India
and other recourse poor
settings.
If sweat testing facility
is not available, then it
is advised to look for
supportive evidence for CF
(Figure. 1) and if they are
suggestive of CF then start
supportive care and refer
the patients to a center
where sweat testing facility
is available.
Management
The first and foremost
step is early diagnosis.
A comprehensive care
of children with CF
Continued on page 15

14 RespiMirror|Volume V, Issue V, November - December 2015|

>>Continued from page 14 Cystic Fibrosis in India
needs a multi- disciplinary team consisting
of physician, physiotherapist,dietician,
microbiologist, psychologist and social
scientist. The principles of management
include: pancreatic enzyme replacement,
supplementation of fat soluble vitamins,
salt supplementation, airway clearance,
antibiotics and supportive care.
Though most pharmacologic agents
required for treatment of CF are available
in India but there is lack of experienced
physicians and other members of the team.
There is a need to create awareness about
CF and train physicians as well as other
supporting team members.
Pancreatic enzyme replacement: Enteric
coated tablets or spherules are administered
with each feed/ meal in doses of 2-3000 IU
of lipase. Doses are adjusted by observing
number of stools, smell of stool and weight
gain of the child. It should not exceed 10000
IU of Lipase /Kg/day.
Supplementation of fat soluble
vitamins: Due to pancreatic origin stetorrhea
children with CF develops deficiency of fat
soluble vitamins (Vitamin A, D, E, K). All
children with CF should receive two times
of the recommended daily allowance of these
vitamins. Care should be taken that vitamin
preparations are taken with enzymes.
Therefore it is recommended that they can
take vitamins with meals.
Salt supplementation: Due to excessive
Figure 1. Suggested algorithm for diagnosis and management of cystic fibrosis in resource limited setting
Sweat test facility available
Sweat chloride estimation
High (> 60 mEq/L)
Repeat
Repeat also > 60 mEq/L
Treat as CF
Follow up as suspected CF, start empirical enzyme replacement, vitamin supplements and chest physiotherapy
Give diagnosis of confirmed CF only after getting sweat test or mutational analysis from centers where this
sweating during summer, children with
CF may lose more salt and therefore get
dehydration. They should be supplemented
with potassium chloride and allowed to take
salt adlib, specifically during summers.
Airway clearance: Sputum in children
with CF is thick and sticky and is responsible
for airway obstruction and subsequent
damage leading to bronchiectasis. Therefore
it is important to keep the airways clear of
secretions by maintaining good hydration,
salt supplementation, using nebulised
hypertonic saline inhalation and aggressive
physiotherapy.
Antibiotics: Due to thick and viscid
airway secretions, children with CF get
infections with usual organisms in the
beginning but ultimately get colonized
with pseudomonas species. Colonization
with pseudomonas species is responsible
for respiratory morbidities. Therefore, it is
treated aggressively with systemic antibiotics
when detected first time and during
pulmonary exacerbations. Long term inhaled
antibiotics (Tobramycin/ colistin etc) should
be used to keep pseudomonas species under
control and this has been shown to improve
pulmonary outcomes in children with CF.
Supportive care: To maintain nutritional
status children are encouraged to eat
almost one and half to two times the daily
caloric requirement. If they are not able to
take adequate calories, they can be fed by
nasogastric tube or gastrostomy. Children
Suspect cystic fibrosis
(Recurrent or persistent pneumonia, frequent bulky stools, Failure to thrive
Sweat test facility NOT available
Borderline (40-60 mEq/L) Low (< 40 mEq/L)
Repeat Not CF
Persistently boderline
Try to get mutational
analysis
facility is available
with CF should be regularly monitored for
growth and complications of other organs,
including delayed puberty, development of
cystic fibrosis related diabetes, chronic liver
disease, etc
Since there is no cure and the available
modalities of treatment is very expensive,
there is a need for counseling of family,
development of self help groups that generates
funds and seeks support from government as
well as non-governmental organization.
Prognosis of CF
Research over past 5-6 decades has
improved the survival of children with CF
from 6 months to 3-4 decades. The survival
has improved with best supportive care. In
India, factors that were found associated
with decreased survival in our patients
include: age at onset of symptoms <2 months,
frequency of pneumonia >4 episodes/year,
severe malnutrition at the time of diagnosis
and colonization with Pseudomonas at the
time of diagnosis. Almost all the factors are
modifiable if we create awareness about CF,
diagnose them early and institute appropriate
treatment. At present no long term survival
data are available from India. Indian CF
services need to go a long way to match with
western countries. However, early diagnosis,
aggressive chest physiotherapy with
judicious use of antibiotics and nutritional
management can improve the quality of
life and survival in CF patients even in
developing countries like India.
Acid base status of blood,
serum sodium, chloride,
potassium, stool for fat
globules and sputum/
respiratory secretion culture
for Pseudomonas
Look for: hypochloremia,
hyponatremia, hypokalemia,
metabolic alkalosis,
numerous fat globules on
stool microscopy,
Pseudomonas species in
respiratory secretions
If>2 abnormalities are
present
|Volume V, Issue V, November - December 2015| 15
>>Continued from page 7 Allergic bronchopulmonary
in an appropriate clinical setting, very high
serum levels of IgE- or IgG-A. fumigatus may
be diagnostic of ABPA.
Precipitating antibodies against A.
fumigatus :
Precipitating antibodies against A.
fumigatus, as demonstrated by the double
immunodiffusion technique of Outcherlony
can be detected in the unconcentrated
serum of 70% of patients. With concentrated
serum, these antibodies can be detected in
92% of patients with a radiological infiltrate.
There may be false positive results in 10% of
asthmatics who do not have ABPA. High levels
of serum precipitins against A. fumigatus
have also been shown in various other forms
of chronic pulmonary aspergillosis.
Sputum examination
Sputum eosinophilia is often present
and fungal hyphae can be demonstrated on
sputum smear examination. Expectoration of
golden brown plugs in sputum as well fungal
culture of the sputum due to its low specificity
has been included in the minor diagnostic
criterion.
Pulmonary function testing:
It is a relatively insensitive test and does
not help to define the extent of disease or
exclude it. Patients in remission can have
normal lung function studies if the asthma
is well controlled, even in the presence of
bronchiectasis. During an acute episode/
exacerbation, PFT may sometimes show,
other than obstruction, a restrictive pattern
with reduction in total lung capacity, vital
capacity, forced expiratory volume in the
first second (FEV1) and impaired diffusion
capacity for carbon monoxide.
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16 RespiMirror|Volume V, Issue V, November - December 2015|
Staging
Five stages of ABPA are (i) acute,
(ii) remission, (iii) exacerbation, (iv)
corticosteroid dependent asthma and (v)
fibrotic lung disease. The staging of the disease
may be done at the time of diagnosis and
may be re-evaluated over a period of time. In
patients with acute disease (stage 1), remission
(stage 2) may occur either spontaneously or
may be induced by treatment. Exacerbations
after a period of prolonged remission are also
known to occur. Stage 4 ABPA is clinically
indistinguishable from corticosteroid
dependent asthma without ABPA. Fibrotic
lung disease in stage 5, ABPA may be
associated with clubbing and cavitation
and can be difficult to differentiate from
fibrocavitary disease caused by tuberculosis.
Treatment:
The goals of treatment of ABPA are: (i) to
detect and treat ABPA exacerbations promptly
so as to prevent or minimise bronchiectasis
that develops at the site of infiltrates, (ii)
manage associated asthma (stage I to IV)
or irreversible lung disease (stage V), (iii)
exclude ABPA in family members, and
(iv) identify a potential environmental
source of the incriminated fungus. Systemic
glucocorticoids and antifungal agents are the
two main group of drugs studied to date. The
usage of inhaled corticosteroids alone may
only help to achieve asthma control but would
not prevent symptomatic exacerbations.
Oral corticosteroids are the mainstay for the
treatment of ABPA. Corticosteroids have a
potent anti-inflammatory property which
helps to suppress the immune hyperreactivity
seen in patients with asthma and ABPA.
In patients with stages 1 (acute) and 3
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Edited by : Ms. Simi Raghavan
(exacerbation) of the disease, the most widely
advocated steroid protocol is prednisolone
(0.5 mg/kg/day for the first 2 weeks followed
by 0.5 mg/kg/day on alternate days for the next
2 months). Once decline in the total serum
IgE level >35% is achieved, the dosage can be
tapered off by 2.5 mg every 2 weeks. Stage 4
ABPA patients are managed with 10-40 mg
prednisolone on alternate days for many
years as repeated attempts to discontinue
may result in unacceptable wheezing. If
prednisolone can be discontinued, the patient
should initially be evaluated every 6-8 weeks
to help determine whether remission (stage
2) is maintained. Stage 5 patients may require
daily prednisolone along with therapy for
corpulmonale and hypoxaemia. Anti-fungals
have a limited role in the management of
ABPA.
It was presumed that by decreasing
the fungal load, there would be reduced
antigenic stimulation and thus a decrease
in the inflammatory response. Itraconazole
has been used as a steroid-sparing agent in
patients on long-term oral prednisolone and
has been the focus of attention in recent years.
With the limited data available currently, use
of itraconazole alone should be restricted
to patients in whom oral corticosteroids are
absolutely contraindicated. ABPA and other
Aspergillus-related disorders : Although the
clinical categories of Aspergillus-associated
respiratory disorders usually remain
mutually exclusive, concomitant occurrence
of ABPA and AAS can be encountered. When
aspergilloma formation occurs in an ABPA
cavity, haemoptysis can be difficult to treat.
We have twice reported concurrent ABPA,
AAS and aspergilloma in a single patient.