Chronic Cough Due to Bronchiectasis: ACCP

Evidence-Based Clinical Practice Guidelines

Mark J. Rosen

Chest 2006;129;122-131
DOI 10.1378/chest.129.1_suppl.122S
The online version of this article, along with updated information
and services can be found online on the World Wide Web at:
http://chestjournal.org/cgi/content/abstract/129/1_suppl/122S

CHEST is the official journal of the American College of Chest

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 Chronic Cough Due to Bronchiectasis
ACCP Evidence-Based Clinical Practice Guidelines
Mark J. Rosen, MD, FCCP

Background: Bronchiectasis is a condition that is characterized by the permanent dilation of

bronchi with destruction of elastic and muscular components of their walls, usually due to acute
or chronic infection. The cardinal symptom is a chronic productive cough.
Methods: Review of articles cited in the systematic literature search, along with others found in
Ovid MEDLINE and the Cochrane Library (including the Cochrane Database of Systematic
Reviews, the Cochrane Controlled Trial Register, and the Database of Abstracts of Reviews of
Effectiveness) from 1966 through 2003.
Results/conclusions: High-resolution CT scanning of the chest is the preferred means of
establishing the diagnosis of bronchiectasis. With the increasing use of antibiotics in the
treatment of childhood infection in the last several decades, an increasing percentage of
patients with bronchiectasis now have an underlying disorder that predisposes them to
chronic or recurrent infection. These include cystic fibrosis, common variable immunodefi-
ciency, HIV infection, primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis,
and chronic Mycobacterium avium complex infection. A variety of agents have been used to
improve cough effectiveness and prevent infectious exacerbations in patients with bronchi-
ectasis, with variable results. Chest physiotherapy offers a modest benefit in increasing
sputum volume, but its long-term effectiveness is unknown. Selected patients with localized
idiopathic bronchiectasis that causes intolerable symptoms despite maximal medical therapy
should be offered treatment with surgery. Patients with exacerbations of bronchiectasis
should be given antibiotics, with the choice of agents depending on the likely causative pathogens.
(CHEST 2006; 129:122S–131S)

Key words: allergic bronchopulmonary aspergillosis; antibiotics; bronchiectasis; bronchodilators; chest physiotherapy; cystic
fibrosis; mucolytics; Mycobacterium avium complex; primary ciliary dyskinesia

Abbreviations: ABPA ⫽ allergic bronchopulmonary aspergillosis; CF ⫽ cystic fibrosis; GERD ⫽ gastroesophageal reflux
disease; HRCT ⫽ high-resolution CT; MAC ⫽ Mycobacterium avium complex; PCD ⫽ primary ciliary dyskinesia;
rhDNase ⫽ recombinant human DNase

B ronchiectasis is a condition characterized by

the permanent dilation of bronchi with de-
struction of the elastic and muscular components
of their walls, usually due to acute or chronic
infection.1,2 Bronchiectasis due to recurrent or
severe infection in patients without an apparent
underlying cause has become less common with
the increasing use of immunization and antibiotics
Reproduction of this article is prohibited without written permission
from the American College of Chest Physicians (www.chestjournal.
org/misc/reprints.shtml).
Correspondence to: Mark J. Rosen, MD, FCCP, Division of
Pulmonary and Critical Care Medicine, Beth Israel Medical
Center, First Ave and 16th St, New York, NY 11021; e-mail:
mrosen@bethisraely.org
in treating childhood infections, and with the
declining incidence of pulmonary tuberculosis.2– 4
In the last few decades, an increasing proportion
of patients with bronchiectasis in developed coun-
tries have been found to have an underlying
disorder (disorders are summarized in Table 1).
Regardless of the etiology, the cardinal symptoms
of bronchiectasis are chronic cough and sputum
production, although some patients may have a
nonproductive cough. Recurrent bacterial coloni-
zation and infection lead to progressive airway
injury that is mediated by neutrophils, T lympho-
cytes, and monocyte-derived cytokines.5,6 The ac-
tions of inflammatory mediators, elastase, and

122S Diagnosis and Management of Cough: ACCP Guidelines

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 Table 1—Disorders That Predispose the Patient to the Development of Bronchiectasis

Disorders Description

Focal distribution
Bronchial obstruction Foreign body
Tumor
Broncholithiasis
Compression by peribronchial lymph nodes
Previous pneumonia
Diffuse distribution
CF
Reduced host immunity Congenital and acquired hypogammaglobulinemia (especially IgG and/or IgG subclasses)
HIV infection
Primary ciliary dyskinesia
Allergic bronchopulmonary mycoses
Chronic MAC infection
Aspiration or toxic inhalation
Rheumatoid arthritis
Inflammatory bowel disease
Other congenital disorders ␣1-antitrypsin deficiency
Tracheobronchomegaly (Mounier-Kuhn syndrome)
Cartilage deficiency (Williams-Campbell syndrome)
Young syndrome
Pulmonary sequestration
Marfan syndrome
Yellow nail syndrome

collagenase lead in turn to inflammation and the Materials and Methods

destruction of the elastic and muscular compo-
nents of bronchial walls, while the contractile
force of the surrounding lung tissue exerts trac-
tion, expanding the diameter of the involved air-
ways.7,8 Bronchiectasis is also associated with in-
creased bronchial arterial proliferation and
arteriovenous malformations, predisposing some
patients to recurrent hemoptysis.9
Although bronchiectasis can be classified radio-
graphically and pathologically into cylindrical, var-
icose, and cystic varieties,1 these distinctions are
not helpful in determining the etiology, treatment,
and prognosis in individual patients.10 Bronchiec-
tasis may have a focal or diffuse distribution.2
Focal bronchiectasis may follow a severe case of
pneumonia, but with increasing use of antibiotics
in recent years it is more often caused by bronchial
obstruction by a foreign body, broncholith, a
slowly growing tumor, anatomic distortion follow-
ing lobectomy, or enlarged peribronchial lymph
nodes. These predispose the patient to recurrent
infection and bronchiectasis distal to the site of
obstruction. Focal bronchiectasis due to bronchial
obstruction is important to recognize because it
may be amenable to local intervention with bron-
choscopic procedures or surgery. Diffuse bronchi-
ectasis is usually caused by an underlying disorder
(Table 1).
The articles produced by the Duke systematic literature search
were reviewed (see section 2 of this guideline), along with others
found in searches of Ovid MEDLINE and the Cochrane Library
(including the Cochrane Database of Systematic Reviews, the
Cochrane Controlled Trial Register, and the Database of Ab-
stracts of Reviews of Effectiveness) from 1966 through 2003.
Diagnosis
Although chronic productive cough is the cardi-
nal feature of bronchiectasis, this symptom occurs
far more commonly in patients with chronic bron-
chitis, asthma, upper airway cough syndrome due
to a variety of rhinosinus diseases, previously
referred to as postnasal drip syndrome, and gas-
troesophageal reflux disease (GERD).11,12 In a
prospective, before-and-after intervention trial,12
a cohort of 71 immunocompetent adults who
complained of expectoration of ⬎ 30 mL of spu-
tum per day underwent a detailed protocol-driven
diagnostic evaluation; upper airway cough syn-
drome was the cause in 40% of patients, asthma in
24% of patients, GERD in 15% of patients, bron-
chitis in 11% of patients, and bronchiectasis in
only 4% of patients. Unusual causes of chronic
cough with excessive sputum production may be
clinically unsuspected initially; these include non-
asthmatic eosinophilic bronchitis13 and bacterial

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suppurative airway disease.14 In the latter disor-
der, patients do not have excessive sputum expec-
toration and may not have radiographic evidence
of bronchiectasis, but have copious purulent se-
cretions found on bronchoscopy.
Excessive sputum volume, purulence, and tenacity
are nonspecific findings. The physical examination
findings in patients with bronchiectasis may reveal
variable degrees of rhonchi, crackles, and clubbing,
or they may be normal. Also, the presence or
absence of crackles on auscultation of the chest does
not correlate with the presence of bronchiectasis as
diagnosed by high-resolution CT (HRCT) scanning
of the chest.4 Sputum cultures are often positive for
Haemophilus influenzae, Staphylococcus aureus,
Streptococcus pneumoniae, and Pseudomonas
aeruginosa, but these pathogens may also be found
in the airways of patients with chronic bronchitis.15,16
The isolation of mucoid strains of Pseudomonas
suggests the diagnosis of cystic fibrosis (CF), isola-
tion of Aspergillus suggests allergic bronchopulmo-
nary aspergillosis (ABPA), and isolation of Mycobac-
terium avium complex (MAC) suggests chronic
infection with that organism, but isolating these
organisms is not specific for these disorders.
Because bronchiectasis is defined as abnormal
dilation of airways, the diagnosis depends on visual-
izing the typical changes either radiographically or
anatomically. Bronchiectasis is sometimes obvious
on routine chest radiographs, but the diagnosis is
usually established using HRCT scanning.17,18 This
test compares favorably with bronchography, which
had been considered a “gold standard,” but bron-
chography is now rarely performed because it is
invasive, unpleasant, and risky, and because the
HRCT scan is as accurate in diagnosis, with sensitiv-
ity and specificity exceeding 90%.
The key feature of bronchiectasis on HRCT scans
is enlarged internal bronchial diameter, where the
bronchi appear larger than the accompanying artery
(called signet ring sign).19 Other HRCT scan find-
ings in bronchiectasis include the failure of the larger
airways to taper while progressing to the lung pe-
riphery, air-fluid levels in dilated airways, and the
identification of airways in the extreme lung periph-
ery. Indirect HRCT scan signs of bronchiectasis
include bronchial wall thickening, mucoid impac-
tion, and focal air-trapping.
Recommendation
1. In patients with suspected bronchiectasis
without a characteristic chest radiograph find-
ing, an HRCT scan should be ordered because
it is the diagnostic procedure of choice to con-
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firm the diagnosis. Level of evidence, low; benefit,
substantial; grade of recommendation, B
Specific Causes of Bronchiectasis
Many cases of bronchiectasis result from perma-
nent bronchial wall damage after a childhood respi-
ratory infection, such as bacterial pneumonia, per-
tussis, or measles. In patients with healed
tuberculosis or other fibrosing lung diseases (eg,
fungal infection, sarcoidosis, or idiopathic pulmonary
fibrosis), traction on major airways from surrounding
fibrotic lung parenchyma may distort their architec-
ture, leading to recurrent infection and traction
bronchiectasis.20 A systematic search for an underly-
ing cause of bronchiectasis often yields a diagnosis
that is amenable to treatment. In a study21 of 150
adults with bronchiectasis, one or more causative
factors were identified in 47% of cases, and the
diagnosis had important prognostic or therapeutic
significance in 15% of cases. In the latter category,
patients with CF were referred to a specialty clinic,
patients with common variable immunodeficiency
were started on Ig replacement therapy, patients
with ABPA were given a trial of corticosteroid
treatment, and a patient with recurrent aspiration
associated with GERD was treated with an intensive
regimen of gastric acid suppression.
Recommendation
2. In patients for whom there is no obvious
cause, a diagnostic evaluation for an underlying
disorder causing bronchiectasis should be per-
formed, because the results may lead to treat-
ment that may slow or halt the progression of
disease. Level of evidence, low; benefit, substantial;
grade of recommendation, B
CF
Occurring in 1 per 2,000 to 3,000 live births, CF is
probably the most common identifiable cause of
bronchiectasis in the United States and Europe. A
mutation of the gene that encodes for the CF
transmembrane regulator protein disrupts the nor-
mal flow of fluid and electrolytes across cell mem-
branes, altering the composition of secretions in the
respiratory tract, pancreas, GI tract, and sweat
glands.22 In the lung, this is manifested by excessive
and tenacious mucus secretion, impaired mucus
transport, dilation and hypertrophy of bronchial
glands, and goblet cell hyperplasia, leading to prox-
imal airway plugging and bronchiectasis. This diag-
Diagnosis and Management of Cough: ACCP Guidelines
nosis should be considered in children and younger (almost always in the dynein arms) and function.29
adults with recurrent sinopulmonary infection and/or Absent or discoordinated movement of cilia in the
bronchiectasis, even in the absence of GI symptoms. respiratory tract leads to recurrent sinus, ear, and
The sweat test (called pilocarpine iontophoresis) is lung infections, which in turn leads to bronchiectasis.
still the most accurate way to diagnose CF, as the The name Kartagener syndrome refers to the triad of
result is positive in almost all cases when performed situs inversus totalis, bronchiectasis, and either nasal
properly.23 Testing for the CF gene is less sensitive, polyps or recurrent sinusitis, occurring in approxi-
as there are ⬎ 700 mutations identified; commercial mately half of patients with primary ciliary dyskinesia
laboratories identify around 90% of CF mutations in (PCD).30 Male patients with PCD are almost always
the general population. infertile due to immotile sperm.29 The disorder is
diagnosed by assessing ciliary motility in semen
samples or in bronchial wall or nasal biopsy speci-
Reduced Host Immunity mens, or by electron microscopy that shows abnor-
mal cilia morphology. In a study29 of 78 patients with
Patients with congenital and acquired hypogam-
PCD diagnosed using a combination of compatible
maglobulinemia (especially involving IgG and/or IgG
clinical features and together with tests of ciliary
subclasses) have an increased frequency of recurrent
ultrastructure and function, chronic rhinitis/sinusitis
rhinosinusitis, respiratory infections, and bronchiec-
occurred in all patients, recurrent otitis media oc-
tasis.24 Hypogammaglobulinemia is diagnosed by
curred in 95% of patients, neonatal respiratory dis-
measuring the serum levels of IgG and IgG sub-
ease occurred in 73% of patients, and situs inversus
classes, along with those of IgM and IgA. In primary
occurred in 55% of patients. All patients had defects
hypogammaglobulinemias, the IgG level is typically
in the ciliary structure, two thirds of which occurred
⬍ 5 g/L, and the IgA level is ⬍ 0.1 g/L; antibody
in the outer dynein arm. Nasal nitric oxide produc-
deficiency can be confirmed by documenting de-
tion was very low compared with that in healthy
creased antibody production to two or more vac-
subjects and may be of diagnostic value. H influenzae
cines, including tetanus, diphtheria, measles,
was the most common bacterial isolate found in
mumps, and pneumococcal vaccine. Patients with
sputum, but Pseudomonas isolates were also com-
IgG deficiency may benefit from IV Ig replacement,
mon. Although the isolation of mucoid strains of
which is associated with a reduced frequency of
Pseudomonas in sputum is considered to be a
infection and a slowing of the progression of bron-
marker of CF, it was found in 15% of subjects with
chiectasis.25,26 Even though this therapy is consid-
PCD.
ered to be the standard of care for primary immu-
nodeficiency diseases with hypogammaglobulinemia,
there are no double-blind placebo-controlled trials
evaluating threshold Ig levels for starting treatment, ABPA
the effect of the standard dose of IV Ig (400 mg/kg
Immune-mediated inflammation in response to
every 28 days) on outcomes, and whether higher
inhaled Aspergillus fumigatus antigen causes this
doses are more effective.26 Those with isolated IgG
disorder in some patients with chronic asthma or
subclass deficiency and recurrent infection may ben-
CF.31 When a similar disorder is caused by other
efit from therapy with IV Ig, but this has not been
species of Aspergillus or other fungi, the term aller-
investigated in controlled clinical trials.26
gic bronchopulmonary mycosis may be preferable.32
Patients with HIV infection are also predisposed
The essential diagnostic criteria for the diagnosis of
to developing recurrent sinopulmonary infections
ABPA in patients with bronchiectasis are the central
and bronchiectasis, probably because of abnormal
distribution of disease (inner two thirds of chest on
B-lymphocyte function.27,28 The treatment of pa-
CT scan), asthma, immediate cutaneous reactivity to
tients with HIV infection and bronchiectasis with IV
Aspergillus antigen, elevated total serum IgE level
Ig has not been studied systematically. The impact of
(⬎ 417 IU/L or 1,000 ng/mL), and elevated serum
combination antiretroviral therapy on the severity
levels of IgG-A fumigatus (Aspergillus precipitins) or
and course of airway disease is also unknown, al-
IgE-A fumigatus in comparison to those seen in sera
though the restoration of immune function would be
from skin-test positive patients who did not have
expected to be beneficial.
ABPA.33 Patients may have pulmonary infiltrates and
eosinophilia, but these findings are not needed to
Primary Ciliary Dyskinesia establish the diagnosis. Mucoid impaction of the
bronchi and bronchocentric granulomatosis are also
This refers to a group of autosomal-recessive associated with ABPA.
disorders associated with defective ciliary structure As the disease is a manifestation of a hypersensi-

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tivity reaction rather than an infection, treatment is
aimed at immune modulation. The administration of
oral prednisone to patients with ABPA is associated
with the improvement of asthma, and the presence
of pulmonary infiltrates and eosinophilia, with reduc-
tion in serum levels of IgE, and probably reduced
progression of bronchiectasis. The optimal dose is
unknown, but 0.5 mg/kg/d is recommended, fol-
lowed by a gradual taper and adjustment based on
the patient’s condition.34 The long-term use of cor-
ticosteroids is often necessary, but carries risk, in-
cluding the development of invasive Aspergillus in-
fection. Therefore, the antifungal agent itraconazole
was tested in patients with ABPA as an adjunctive,
steroid-sparing agent in two randomized placebo-
controlled studies.35,36 Both studies spanned 16
weeks of treatment and showed reduced levels of
markers of systemic immune activation (serum IgE
level and eosinophil count). The study by Wark et
al36 also showed reduced levels of markers of airway
inflammation in induced sputum. Neither study35,36
showed significant changes in lung function, al-
though the study by Wark et al36 showed that
subjects receiving itraconazole experienced fewer
exacerbations of disease requiring increased doses of
corticosteroids. Therefore, while itraconazole ap-
pears to be promising as adjunctive treatment for
patients with ABPA, long-term trials are needed to
assess the clinical efficacy and safety in patients in
different disease severity strata. There have been no
randomized controlled trials to evaluate the use of
antifungal therapies in patients with ABPA compli-
cating CF.37
MAC Infection
A clinicopathologic subtype of chronic pulmonary
infection with this pathogen occurs predominantly in
patients who are white women in their seventh or
eighth decade of life with no underlying immune
compromise (called the Lady Windemere syn-
drome.)38,39 It is unknown why this group is at
increased risk, but an undefined inherited or ac-
quired immune deficiency state is presumed to exist.
These patients typically present with chronic cough,
which is often associated with fever and weight loss.
The symptoms are insidious, and there is often an
interval of several years between the onset of illness
and the diagnosis. Radiographs and CT scans show
bronchiectasis and nodular densities, especially in
the middle lobe and lingula.17 The nodules are
typically distributed around peripheral vessels and
airways, frequently with a tree-in-bud configuration.
In the past, these patients were thought to have
preexisting bronchiectasis and “colonization” with
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MAC, but the presence of granulomas in the airways
and the response to antimycobacterial therapy indi-
cates that MAC infection is the primary disorder that
leads to progressive airway damage and bronchiec-
tasis. The diagnostic criteria for pulmonary infection
with MAC have been reviewed elsewhere and are
based on expert opinion.40 Sputum cultures are not
sufficiently sensitive to establish the diagnosis in
many patients.41 In patients with typical clinical and
radiographic features of MAC infection, it is diag-
nosed in some patients with bronchoscopic biopsies,
and other patients are treated empirically. There
have been no large randomized trials of the treat-
ment of MAC-associated lung disease, but based on
in vitro data, there is expert consensus that treat-
ment with a macrolide (eg, clarithromycin or azithro-
mycin) with ethambutol and a rifamycin (eg, rifabu-
tin or rifampin) constitute first-line therapy for
patients with severe or progressive symptoms.40
However, these regimens are prolonged and often
poorly tolerated, and patients often relapse.
Other Disorders
Up to 3% of patients with rheumatoid arthritis
have symptomatic bronchiectasis, and up to 30%
have HRCT-diagnosed disease.42 Some have recom-
mended testing for serum rheumatoid factor in
patients with bronchiectasis with no defined etiology.
Inflammatory bowel disease, and especially chronic
ulcerative colitis, is associated with recurrent respi-
ratory tract infection and bronchiectasis.43 Mecha-
nisms may include airway infiltration by immune
effector cells, enhanced autoimmunity, and compli-
cations of immune-modulating therapy.2 A variety of
congenital disorders listed in Table 1 predispose the
patient to chronic respiratory infection and bronchi-
ectasis; these have been discussed in detail else-
where.44
Treatment
In addition to the treatment of an identified
underlying disease, pharmacotherapy may be used
to enhance bronchodilation and improve mucocili-
ary clearance, antibiotics may be used to prevent
and treat recurrent infection, maneuvers may be
designed to mobilize secretions (eg, chest physio-
therapy), and mucolytic agents and occasionally
surgery may be used to treat localized disease. The
goal of treatment is generally to improve the
symptoms of cough, sputum production, and dys-
pnea, and to prevent the progression of airway
damage. Evaluating the effects of treatment in
Diagnosis and Management of Cough: ACCP Guidelines
patients with bronchiectasis is limited by study
methodology. Randomized trials of these treat-
ments generally lack proper control groups, and
the surrogate end point of “effectiveness” is usu-
ally the volume of sputum production or the
clearance of radiolabeled aerosol from the lung.
More meaningful studies should focus on the
number of exacerbations over time and on mea-
sures of health-related quality of life.
Treatment of Stable Bronchiectasis
Bronchodilators
Bronchodilators, including short-acting and long-
acting ␤-agonists, anticholinergics, leukotriene an-
tagonists, and theophylline are useful in the manage-
ment of COPD and/or asthma. Because many
patients with bronchiectasis also have COPD, and
many show signs of airflow obstruction and bronchial
hyperreactivity, patients commonly receive broncho-
dilator therapy. However, there have been no ran-
domized studies that have validated their usefulness
in the management of cough, sputum production,
dyspnea, or other end points in patients with bron-
chiectasis.45– 48
Recommendation
3. In patients with bronchiectasis with air-
flow obstruction and/or bronchial hyperreactiv-
ity, therapy with bronchodilators may be of
benefit. Level of evidence, expert opinion; benefit,
small; grade of recommendation, E/C
Mucolytics
The purpose of mucolytic drugs is to assist
tracheobronchial clearance by altering the proper-
ties of sputum. Of this class of agents, only
recombinant human DNase (rhDNase) was stud-
ied in randomized controlled trials in stable pa-
tients with bronchiectasis not caused by CF.49 –51
DNA release by neutrophils in airways increases
sputum viscosity, and rhDNase administered by
aerosol digests DNA, thereby decreasing sputum
viscosity and hopefully mucus plugging, infection,
and inflammation. This agent was not associated
with significant benefit in these two trials, and
cannot be recommended for patients with idio-
pathic bronchiectasis. However, patients with CF
treated with rhDNase enjoyed spirometric im-
provement for up to 2 years compared with pla-
cebo, with a nonsignificant reduction of risk of
infectious exacerbations.52
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Recommendation
4. In patients with bronchiectasis caused by
CF, rhDNase should be used to improve spi-
rometry. Level of evidence, low; benefit, small;
grade of recommendation, C
Antiinflammatory Agents
Inhaled corticosteroids are used commonly to
treat bronchial inflammation and to prevent exacer-
bations of asthma and COPD. Two randomized
short-time (4 and 6 weeks, respectively) placebo-
controlled trials53,54 of inhaled corticosteroids in
patients with idiopathic bronchiectasis showed non-
significant trends toward improved FEV1, FVC,
diffusing capacity of the lung for carbon monoxide,
and residual volume, but no effect on toal lung
capacity, sputum production, cough, wheeze, or
dyspnea. Oral corticosteroids may be more effective
in treating bronchial inflammation, but there have
been no randomized controlled trials addressing
clinical end points in patients with bronchiectasis,
other than when it is associated with known cortico-
steroid-responsive conditions like asthma, COPD,
rheumatoid arthritis, and ulcerative colitis.55 How-
ever, the administration of oral corticosteroids at a
prednisolone-equivalent dose of 1 mg/kg on alter-
nate days appears to slow the progression of lung
disease in CF patients, but is associated with signif-
icant side effects, especially in children.56 –59 There-
fore, systemic corticosteroid administration may be
considered in patients with CF, but concerns about
side effects preclude a general recommendation for
its use.
Recommendation
5. In patients with CF, prolonged treatment
with systemic corticosteroids should not be of-
fered to most patients because of significant
side effects. Level of evidence, low; benefit, con-
flicting; grade of recommendation, I
Similarly, the administration of ibuprofen to pa-
tients with CF with the goal of reducing inflamma-
tion showed modest benefits in patients with mild
disease, but its use also cannot be recommended at
this time because of the potential of side effects with
prolonged use.60
Recommendation
6. In patients with CF, prolonged courses of
ibuprofen should not be used. Level of evidence,
low; benefit, conflicting; grade of recommendation, I
CHEST / 129 / 1 / JANUARY, 2006 SUPPLEMENT 127S
Antibiotics
The systemic and inhaled administrations of
antibiotics were investigated as ways to prevent
exacerbations of bronchiectasis by breaking the
putative vicious cycle of bacterial colonization
leading to inflammation and further airway injury.
A literature review by the Cochrane group re-
vealed six randomized controlled trials61 in pa-
tients with idiopathic bronchiectasis, but these
studies had differences in design that limited the
ability to generalize the outcomes. In general, they
suggested that long-term therapy with antibiotics
is effective for reducing sputum volume and pu-
rulence, but has limited impact on the frequency
of exacerbations and the natural history of the
condition, and may facilitate the emergence of
resistant organisms.
Recommendation
7. In patients with idiopathic bronchiectasis,
the prolonged systemic administration of anti-
biotics may produce small benefits in reducing
sputum volume and purulence, but may also be
associated with intolerable side effects. Level of
evidence, low; benefit, conflicting, grade of recom-
mendation, I
A randomized placebo-controlled trial62 of inhaled
tobramycin delivered by jet nebulizer in patients
with CF showed that it was well tolerated, and was
associated with improved pulmonary function, de-
creased density of P aeruginosa in sputum, and
decreased risk of hospitalization.
Recommendation
8. In patients with CF, therapy with aerosol-
ized antipseudomonal antibiotics are recom-
mended. Level of evidence, low; benefit, interme-
diate; grade of recommendation, C
However, a similar trial63 in patients with bronchi-
ectasis not caused by CF was less encouraging;
although the treatment group had decreased density
of P aeruginosa and was judged more likely to have
an “improved medical condition,” they were also
more likely to report increased cough, dyspnea,
wheezing, and noncardiac chest pain.
Recommendation
9. In patients with idiopathic bronchiectasis,
aerosolized antibiotics should not be used. Level
of evidence, low; benefit, negative; recommendation, D
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Bronchopulmonary Hygiene
A variety of manual and mechanical interventions,
including chest percussion, vibration, postural drain-
age, mechanically assisted cough, and airway oscilla-
tion, are used commonly to facilitate the expectora-
tion of mucus. These maneuvers are also referred to
as chest physiotherapy, and formerly as pulmonary
toilet. Their primary role is to facilitate the clearance
of lower airway secretions in conditions associated
with the hypersecretion of mucus and the inability to
expectorate effectively, as in patients with bronchi-
ectasis. The role of bronchopulmonary hygiene in
treating bronchiectasis and chronic bronchitis is
discussed in a later section of this guideline. Al-
though these techniques are considered to be main-
stays in the treatment of bronchiectasis, they have
modest effects on increasing sputum volume, and
the long-term effectiveness of these interventions is
unknown.
Recommendation
10. In patients with conditions associated
with the hypersecretion of mucus and the in-
ability to expectorate effectively, chest physio-
therapy should be used and patients should be
monitored for symptom improvement. Level of
evidence, expert opinion; benefit, small/weak; grade
of recommendation, E/C
Surgery
There have been no randomized controlled stud-
ies comparing surgery with more conservative man-
agement, and the literature consists of retrospective
reviews of case series. With proper patient selection,
surgery is associated with excellent outcomes and
low perioperative mortality rates.64,65 Surgery to
resect the bronchiectatic lung should be limited to
patients with local disease who have not responded
to medical therapy. Double-lung transplantation is
commonly offered to patients with CF.
Recommendation
11. In selected patients with localized bron-
chiectasis that causes intolerable symptoms de-
spite maximal medical therapy, surgery should
be offered. Level of evidence, low; benefit, substan-
tial; grade of recommendation, B
Treatment of Exacerbations
As bacterial exacerbations of bronchiectasis account
for substantial morbidity, antibiotics are a mainstay of
Diagnosis and Management of Cough: ACCP Guidelines
treatment. Antibiotics are probably effective for exac-
erbations characterized by change in sputum produc-
tion, increased dyspnea, increased cough, fever, in-
creased wheezing, malaise, fatigue, lethargy, reduced
exercise tolerance, reduced pulmonary function, new
pulmonary opacities (although this may represent
pneumonia), and changes in chest sounds.51
Recommendation
12. In patients with exacerbations of bronchi-
ectasis, antibiotics should be used, with the
selection of agents depending on the likely
pathogens. Level of evidence, low; benefit, substan-
tial; grade of recommendation, B
There have been no published randomized place-
bo-controlled trials on the antibiotic treatment of
exacerbations of bronchiectasis since the last Amer-
ican College of Chest Physicians cough guideline,66
and no new recommendations can be offered. Treat-
ment should initially include an agent that is active
against H influenzae and S aureus; patients with
recent antibiotic exposure or CF should be consid-
ered to be at high risk of infection with Pseudomonas
and treated accordingly. Patients should receive at
least 2 weeks of antimicrobial therapy; sputum cul-
ture and sensitivity testing should be performed in
patients who do not have an adequate response, and
they should guide the choice of a longer course of an
oral agent, or even prolonged IV antibiotic therapy.2
Conclusions
Bronchiectasis is diagnosed in approximately 4%
of patients with chronic cough. HRCT scanning of
the chest is the best test to establish this diagnosis.
Most cases of bronchiectasis in adults are idiopathic,
but in the absence of an obvious cause, a diagnostic
evaluation for an underlying disorder should be
performed, as treatment may slow or halt the pro-
gression of airway disease. Bronchodilator agents,
anticholinergic agents, and oral and inhaled steroids
are often used in stable patients with bronchiectasis
as well as during acute exacerbations despite the lack
of objective evidence of benefit. Antibiotics and
bronchopulmonary hygiene are mainstays in the
treatment of exacerbations, but the prolonged use of
systemic and inhaled antibiotics cannot be recom-
mended to prevent these episodes except in patients
with bronchiectasis caused by CF. Surgery is bene-
ficial in a carefully selected subgroup of patients with
focal disease and intractable symptoms.
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Copyright © 2006 by American College of Chest Physicians
Summary of Recommendations
1. In patients with suspected bronchiec-
tasis without a characteristic chest radio-
graph finding, an HRCT scan should be
ordered because it is the diagnostic proce-
dure of choice to confirm the diagnosis.
Level of evidence, low; benefit, substantial;
grade of recommendation, B
2. In patients for whom there is no obvious
cause, a diagnostic evaluation for an underly-
ing disorder causing bronchiectasis should be
performed, because the results may lead to
treatment that may slow or halt the progres-
sion of disease. Level of evidence, low; benefit,
substantial; grade of recommendation, B
3. In patients with bronchiectasis with air-
flow obstruction and/or bronchial hyperreac-
tivity, therapy with bronchodilators may be of
benefit. Level of evidence, expert opinion; bene-
fit, small; grade of recommendation, E/C
4. In patients with bronchiectasis caused
by CF, rhDNase should be used to improve
spirometry. Level of evidence, low; benefit,
small; grade of recommendation, C
5. In patients with CF, prolonged treat-
ment with systemic corticosteroids should
not be offered to most patients because of
significant side effects. Level of evidence,
low; benefit, conflicting; grade of recommen-
dation, I
6. In patients with CF, prolonged courses
of ibuprofen should not be used. Level of
evidence, low; benefit, conflicting; grade of rec-
ommendation, I
7. In patients with idiopathic bronchi-
ectasis, the prolonged systemic adminis-
tration of antibiotics may produce small
benefits in reducing sputum volume and
purulence, but may also be associated
with intolerable side effects. Level of evi-
dence, low; benefit, conflicting, grade of rec-
ommendation, I
8. In patients with CF, therapy with aero-
solized antipseudomonal antibiotics are rec-
ommended. Level of evidence, low; benefit,
intermediate; grade of recommendation, C
9. In patients with idiopathic bronchiec-
tasis, aerosolized antibiotics should not be
used. Level of evidence, low; benefit, negative;
recommendation, D
10. In patients with conditions associated
with the hypersecretion of mucus and the
inability to expectorate effectively, chest
physiotherapy should be used and patients
CHEST / 129 / 1 / JANUARY, 2006 SUPPLEMENT 129S

should be monitored for symptom improve-
ment. Level of evidence, expert opinion; bene-
fit, small/weak; grade of recommendation, E/C
11. In selected patients with localized-
bronchiectasis that causes intolerable symp-
toms despite maximal medical therapy, sur-
gery should be offered. Level of evidence,
low; benefit, substantial; grade of recommenda-
tion, B
12. In patients with exacerbations of
bronchiectasis, antibiotics should be used,
with the selection of agents depending on
the likely pathogens. Level of evidence, low;
benefit, substantial; grade of recommendation, B
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Chronic Cough Due to Bronchiectasis: ACCP Evidence-Based Clinical
Practice Guidelines
Mark J. Rosen
Chest 2006;129;122-131
DOI 10.1378/chest.129.1_suppl.122S
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