MEDICINE
REVIEW ARTICLE
Bronchiectasis—Diagnosis and Treatment
Jessica Rademacher, Tobias Welte
SUMMARY
Background: Radiologically evident bronchiectasis is seen in 30% to 50% of
patients with advanced chronic obstructive pulmonary disease (COPD). As
COPD is now becoming more common around the world, bronchiectasis is as
well.
Methods: We review pertinent articles published before May 2011 that were
retrieved by a selective PubMed search.
Results: The principles of treatment of bronchiectasis in patients who do not
have cystic fibrosis (“non-CF bronchiectasis”) are derived from the treatment
of other diseases: secretolytic and anti-infectious treatment are given as in
cystic fibrosis, while anti-obstructive treatment is given as in COPD. The
few randomized trials of treatment for non-CF bronchiectasis that have been
completed to date do not permit the formulation of any evidence-based recom-
mendations. Many potential treatments are now under evaluation. Hypertonic
saline is often used because of its demonstrated benefit in CF, even though no
benefit has yet been shown for non-CF bronchiectasis. Phase II trials of inhaled
mannitol have yielded promising results, leading to phase III trials that are now
underway. There may be a future role for inhaled antibiotics, particularly in
patients colonized with Gram-negative pathogens. Inhaled tobramycin and
colistin are well established in clinical practice, though not approved for non-
CF bronchiectasis; clinical trials of aztreonam, ciprofloxacin, and gentamicin
are ongoing. Macrolides seem to bring an additional benefit, though the studies
that documented this involved only small numbers of patients. Long-term treat-
ment with inhaled antibiotics and/or macrolides is indicated only if a benefit is
seen within three months of the start of treatment (less sputum, no exacer-
bations).
Conclusion: A national registry of patients with bronchiectasis should be estab-
lished to help us gain better knowledge of its prognostic factors and treatment
options.
►Cite this as:
Rademacher J, Welte T: Bronchiectasis—diagnosis and treatment.
Dtsch Arztebl Int 2011; 108(48): 809–15. DOI: 10.3238/arztebl.2011.0809
Klinik für Pneumologie, Medizinische Hochschule Hannover:
Prof. Dr. med. Welte, Dr. med. Rademacher
Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15
he term bronchiectasis describes a permanent
T dilation of the bronchi and bronchioles as a result
of destruction of the muscles and elastic connective
tissues. The disorder mostly starts with a narrowing of
the bronchial tree triggered by an infection, which may
lead to destruction of the epithelium if it becomes
chronic. The disruption of the mucociliary clearance
results in retention of secretions and predestines the
patient for further infections.
In the past, bronchiectasis mostly had infectious
causes, such as epidemics of pertussis, measles, and
influenza. Today’s most common cause in developing
countries is the postinfectious route. The development
of antibiotic treatments and vaccines has resulted in a
continuous decrease in the number of cases of bron-
chiectasis with postinfectious causes in industrial coun-
tries. Currently, congenital causes of bronchiectasis are
seen more observed than postinfectious causes.
In Europe, bronchiectasis is common in patients with
cystic fibrosis (CF) (1). However, in this review article
we focus on patients with bronchiectasis in whom
cystic fibrosis was excluded (non-CF bronchiectasis).
This article aims to provide an overview over what
is currently known from studies about the diagnostic
evaluation and therapy of this heterogeneous pathology.
Method
We conducted a selective literature search on PubMed.
Relevant articles published before May 2011 were
included in this review.
Incidence and prevalence
Because high-resolution computed tomography
(HRCT) scanning is more commonly used nowadays,
bronchiectasis is diagnosed earlier and at earlier stages.
This has resulted in a seeming increase in the preva-
lence of bronchiectasis. The question of whether the
increase in the numbers of cases is caused by the ageing
population and the increase in chronic lung disorders
will have to remain unanswered.
Very few data on prevalence are currently available.
In New Zealand, the prevalence was reported to be
3.7/100 000 population; in the United States, the rate
was reported to be as high as 52/100 000 (2). Different
diagnostic methods (clinical versus CT) and a different
selection of patients may be responsible for this
discrepancy.
809
MEDICINE

TABLE 1 histologically shows notable metaplasias of the squa-

mous epithelium, although an increased incidence in
Etiology of bronchiectasis (modified from [7]) malignancies has not been observed.
Category Cause
Postinfectious causes
Postinfectious – Viral
– Bacterial Different respiratory infections can cause bronchiecta-
– Fungal sis, including:
– Atypical mycobacteria ● Pertussis
ABPA ● Gram negative bacteria (Pseudomonas aerugino-
COPD
sa, Haemophilus influenzae)
● Viruses (HIV, paramyxovirus, adenovirus, and
Idiopathic traction – Posttuberculous fibrosis
– Radiation fibrosis
flu)
– Pulmonary fibrosis ● Tuberculosis
Aspiration – Foreign body aspiration
● Atypical mycobacteria.
Bronchiectasis subsequent to infection with Myco-
Obstruction – Benign tumors bacterium avium is a typical finding in Lady Winder-
– Enlarged lymph nodes
mere syndrome. Patients suffering from this syndrome
Amyloidosis are often older, immunocompetent women without a
Celiac disease history of smoking or previous pulmonary pathologies
Yellow nail syndrome (4).
Young’s syndrome
Congenital causes
Immunological defects Primary The most common congenital cause for non-CF bron-
– CVID
– Agammaglobulinemia chiectasis is a primary ciliary dyskinesia (PCD). Insuf-
– Hyper-IgE syndrome ficient ciliary movement results in reduced clearance of
Secondary secretions, triggering an increase in the rate of infec-
– Chemotherapy
– Immunosuppressant therapy tions in turn. In combination with a situs inversus, the
– Tumor resultant pathology is known as Kartagener syndrome.
Congenital defects Anatomical Its prevalence is 1/20 000.
– Scoliosis A more recently discovered congenital cause is a
– Marfan syndrome mutation of the ENaC gene, which results in a defective
– Tracheobronchomegaly
Others epithelial sodium channel. A hyperactive sodium chan-
– Primary ciliary dyskinesia nel triggers a disturbance to the salt and water homeo-
– Kartagener syndrome stasis of the respiratory mucosa (5).
– Alpha-1-antitrypsin deficiency
– defect ENaC protein
Chronic obstructive pulmonary disease
Diffuse panbronchiolitis
Patients with advanced chronic obstructive pulmonary
Rheumatoid arthritis disease (COPD) may have bronchiectasis; the literature
Systemic lupus erythematodes reports rates between 30% and 50% (6). These patients
Chronic bowel disease more often suffer from dyspnea and show poorer lung
function (6). CT-morphologically, bronchiectasis in
ABPA, allergic bronchopulmonary aspergillosis; COPD, chronic obstructive pulmonary disease; COPD differs from classic bronchiectasis, since the
ENaC, epithelial sodium channel; CIVD, common variable immunodeficiency ectasis is less pronounced but the peribronchial infil-
tration is more pronounced. With the rising global
prevalence of COPD, bronchiectasis is of increasing
importance.
Pathophysiology and etiology
Different mechanisms (Table 1) lead to the develop- Clinical features
ment of bronchiectasis, but the pathophysiological end Patients with bronchiectasis complain of chronic
stage is similar. In the beginning, patients usually have cough, sputum production, and lethargy. Hemoptysis,
damaged bronchial epithelium as a result of inflam- chest pain, weight loss, bronchospasm, dyspnea, and
mation; the surrounding parenchyma is infiltrated by impaired physical performance have also been ob-
inflammatory cells (Figure 1). The destruction of the served (7). The often mentioned three-layer sputum
neighboring tissues results in dilation in the form of consisting of a foamy upper layer, mucous middle
cylindrical, varicose, or cystic distensions with destruc- layer, and viscous purulent bottom layer is pathogno-
tion of the surrounding structures. This will in turn lead monic, but does not always occur. Some patients are
to deficient mucociliary clearance. The result is reten- symptom free in everyday life and become clinically
tion of secretions, which in turn attracts bacterial colo- conspicuous only during exacerbations.
nization with chronic inflammation (3). Furthermore, a Many patients have regular exacerbations, the aver-
thickening of the bronchial mucosa will ensue, which age is 1.5 per year. Exacerbation is defined as the

810 Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15
 MEDICINE

presence of four or more of the symptoms listed in Box

1 (8). The loss of lung function in non-smokers with
bronchiectasis has been reported to be about 50 mL/
year. Factors that would imply disease progression are
frequent exacerbations, chronic colonization with
Pseudomonas aeruginosa, and confirmed systemic
inflammation. In case of severe bronchiectasis, pul-
monary hypertension and systolic and diastolic left
ventricular dysfunction may develop.

Diagnostic evaluation
Investigating the colonization
Microbiological sputum analysis is a standard diag-
nostic procedure. Risk factors for colonization include
varicose or cystic bronchiectasis, a forced expiratory
volume in one second (FEV1) <80%, and age <14
years at first diagnosis (7). The most common
pathogens are Haemophilus influenzae, Pseudomonas
a
spp, and Streptococcus pneumoniae. In progressive dis-
ease with recurring exacerbations and negative sputum
results, bronchoscopy is indicated for the purpose of
specimen sampling.

Imaging
The imaging method of choice is high-resolution com-
puted tomography (Figure 2). Often the type and local-
ization of the radiological changes can provide indi-
cations of the pathogenesis. Bronchiectasis in the
proximal airways is typical of allergic bronchopulmo-
nary aspergillosis; multiple nodular bronchiectasis may
indicate infection with Mycobacterium avium complex.

Therapy
The treatment for bronchiectasis is mostly based on ex-
periences gained from the treatment of COPD and CF
(Figure 3). Whether these concepts actually translate
has not been studied. Only few controlled studies exist,
so that for non-CF bronchiectasis, hardly any evidence-
based recommendations can be made. The aims of
treatment for bronchiectasis are:
● Treating the underlying disease
● Improving mucociliary clearance or drainage
ofsecretions
● Treating the infection
● Treating airway obstruction
● Treating the chronic inflammation that leads to
disease progression.
Treating the underlying disease
If possible, the underlying disease should be treated
first. This primarily applies to immunodeficiency
syndromes. In hypogammaglobulinemia, substitution
treatment with immunoglobulins can be given (0.4 g/kg
body weight every 4–6 weeks).
Draining secretions
Breathing therapy and physiotherapeutic measures are
the basic treatments for bronchiectasis, to improve
drainage of secretions and deal with dyspnea. The
mainstay of treatment is sufficient administration of
b
Figure 1: Bronchiectasis with chronically and acutely inflamed mucosa with accompanying
fibrosis
fluids for secretolytic purposes. This can be supported
by inhalation of hypertonic saline solution. Especially
inhaling hyperosmolar solutions has been found to be
beneficial. The raised salt concentration results in an
osmotic penetration of fluid into the secretions, thus
improving their rheological properties, which in turn
results in faster and more effective clearance. Studies
using 7% saline for inhalation by CF patients have
shown improved lung function and secretion clearance
(9). In non-CF bronchiectasis, reduced sputum viscos-
ity was found in a small number of patients (n=24) dur-
ing a non-exacerbation period (10).
In contrast to other hyperosmolar solutions, manni-
tol has the advantage of a longer half life within the
airways. In an open label, non-controlled study over 12
days, quality of life, lung function, and sputum viscos-
ity were notably improved (11). A disadvantage of
mannitol is the fact that hyperresponsiveness increases
during inhalation. Currently, further studies are being

Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15 811
MEDICINE
BOX 1
Symptoms of exacerbation
● Increase of sputum with cough
● Increased dyspnea
● Raised temperature >38° C
● Increased wheezing
● Lowered physical resilience
● Fatigue
● Deterioration in lung function
● Radiological signs of infection
A minimum of 4 symptoms are the defining criteria for
an exacerbation
(modified from [8])
conducted in order to gain licensing approval for the
substance.
Vaccinations
No randomized studies of vaccinations exist for this
group of patients. The effect of annual flu vaccination
has been proved for other chronic airway disorders,
such as COPD, and translates to patients with bron-
chiectasis. Data from smaller cohort have shown that
the pneumococcal vaccine is beneficial, although a
final conclusion cannot currently be drawn. In prin-
ciple, the recommendation is to follow the vaccination
guidelines issued by Germany’s Standing Vaccination
Committee (STIKO) for patients with chronic pulmo-
nary disorders.
Antibiotics
In an acute exacerbation of bronchiectasis, antibiotics
should be given if an increase in dyspnea and sputum
volume is observed and the sputum has assumed a
yellow-green or green tinge.
If the patient is known to have chronic colonization
with respiratory pathogens, targeted treatment should
be started while taking into consideration the latest
antibiogram. If no microbiological result is available
then a broad spectrum antibiotic should be selected for
initial treatment. This should include treatment for
Pseudomonas strains, since these range among the
pathogens particularly in severely ill patients and deter-
mine the prognosis.
In the outpatient setting, the fluoroquinolones levo-
floxacin or ciprofloxacin are the only available options.
It needs to be borne in mind, however, that ciprofloxa-
cin is not sufficiently effective against Pneumococci,
the most common pathogens in community acquired
pneumonia. Pletz et al. reported the case of a patient
with bronchiectasis in whom consecutive courses of
treatment failed in the presence of ciprofloxacin resis-
tant Pneumococci (e1).
812
Figure 2: Bronchiectasis CT-morphology
In hospital inpatients, the range of substances that
are effective against Pseudomonas spp is wider (carba-
penem, cephalosporins with activity against Pseudo-
monas, ureidopenicillins). Whether combination ther-
apy using a beta-lactam with an aminoglycoside or flu-
oroquinolone is superior to monotherapy with a sub-
stance that is active against Pseudomonas is the subject
of controversial debate. Pseudomonas infections
should be treated for 10–14 days. In patients not at risk
from Pseudomonas aeruginosa, treatment with amin-
openicillin/inhibitor or third-generation cephalosporins
is recommended. The duration of treatment is usually
seven days. Attempts to diagnose the pathogen should
be made before antibiotics are given, and the antibiotic
treatment should be tailored accordingly.
The importance of antibiotics treatment outside
exacerbations is the subject of controversy. Thus far,
attempts to reduce the amount of pathogens by means
of long-term oral antibiotic treatment, and to lower the
rate of exacerbations, have remained unsuccessful. The
development of resistance patterns in patients receiving
long-term therapy may be important in this context.
Inhaled antibiotics are standard treatment for
patients with CF who have been colonized with Pseu-
domonas aeruginosa (12). Since 25% of patients with
non-CF bronchiectasis are colonized with Pseudomo-
nas aeruginosa, this therapeutic principle may offer an
advantage in this setting. In the meantime, smaller
studies have shown the importance of inhaled anti-
biotics in non-CF bronchiectasis. Significant clinical
improvement has been shown, with a reduced density
of pathogens and eradication of Pseudomonas
aeruginosa in up to 35% of cases (13). Patients receiv-
ing treatment with inhaled tobramycin had fewer symp-
toms and an improved quality of life (14). Inhaled
colistin led to improvements in lung function and
quality of life (15); furthermore, a reduction in
inpatient admissions and exacerbations has been
reported (16). Inhaled aztreonam lowered the rate of
Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15

FIGURE 3
Therapeutic scheme for bronchiectasis (modified from [e2])
exacerbations, reduced symptoms, and improved lung
function in patients with CF (17). A study of inhaled
aztreonam in non-CF bronchiectasis is in the planning
stages.
A liposomal preparation of inhaled ciprofloxacin
confirmed the reduced pathogenic load of Pseudomo-
nas aeruginosa (e3); the same was confirmed for
powder inhalation of ciprofloxacin (e4). Further studies
of inhaled amikacin and intratracheal instillation of
fosfomycin/tobramycin are expected.
In a randomized controlled study, inhaled gentamy-
cin led to eradication of Pseudomonas aeruginosa in
30.8% of cases and prolonged the interval to the next
exacerbation (120 days versus 61.5 days) (18). Another
study of inhaled gentamycin found a lowered rate of
exacerbations and improved quality of life (19). Table 2
provides an overview of inhaled antibiotics.
Anti-obstructive therapy
If a patient’s airways are obstructed, anti-obstructive
treatment similar to COPD should be considered. Para-
sympatholytics and beta-sympathicomimetics consti-
tute the treatment of choice. Long-acting substances
(tiotropium bromide or salmeterol/formoterol) seem
superior to short-acting substances. Proof of superiority
is lacking for inhalation therapy with compression or
ultrasound nebulizers (both of which are popular in
Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15
MEDICINE
TABLE 2
Selection of the most researched inhaled antibiotics
Substance Results Source
Tobramycin Eradication in 13–35%, reduction of pathogenic (13)
load, improved lung function (14)
Colistin Rise in FEV1, eradication in 3 of 18 cases, fewer (15)
exacerbations, reduction of pathogenic load (16)
Aztreonam CF: improved lung function, longer interval to (17)
exacerbation
Liposomal cipro- Reduction of pathogenic load (e3)
floxacin Reduction of pathogenic load (e4)
Ciprofloxacin
Gentamycin Fewer exacerbations, improved quality of life (19)
Eradication of P.a. in 30.8 % improved quality of (18)
life
CF, cystic fibrosis; P. a., Pseudomonas aeruginosa;
FEV, forced expiratory volume in one second
Germany) compared with conventional treatment with
metered-dose aerosol inhalers or powder inhalers.
Inhibiting inflammation
Oral corticosteroids are often administered in acute
exacerbations of bronchiectasis, in analogy to acute ex-
acerbations of COPD. For inhaled steroids, long-term
usage seems to confer benefits. Tsang et al. showed in a
study of 73 patients with non-CF bronchiectasis a re-
duction in the exacerbation rate and sputum production
when using inhaled steroids (20). Randomized studies
are, however, lacking.
Macrolide antibiotics such as azithromycin have a
potent anti-inflammatory effect in addition to their anti-
bacterial effects. They reduce the production of proin-
flammatory cytokines. The cytokines act as chemo-
kines for neutrophils and effect an expression of
adhesion molecules, which neutrophils require for their
migration from the bloodstream to the interstitium. Fur-
thermore, in addition to their regular bacteriostatic
effects, macrolides inhibit the production of biofilms
by Pseudomonas aeruginosa (independently of their
antibiotic effectiveness) (21).
In the therapy of neutrophil-dominated, chronic
inflammatory pulmonary disorders, such as diffuse
panbronchiolitis (DPB) or CF, macrolides are already
in use, successfully and without notable side effects.
The treatment with macrolide antibiotics has led to a
reduction in the amount of sputum and improved 5-year
survival in patients with non-CF bronchiectasis too
(22). At this point in time, however, neither inhaled
antibiotics nor macrolides are licensed for the treatment
of patients with non-CF bronchiectasis.
Allergic bronchopulmonary aspergillosis
Allergic bronchopulmonary aspergillosis (ABPA) is a
rare but typical complication in bronchiectasis. The
813
MEDICINE
BOX 2
Diagnostic criteria for allergic
bronchopulmnaory aspergillosis
1. Asthma
2. Skin reactions*1
3. Raised total IgE in serum (> 1 000 ng/L)*1
4. Raised specific IgE and/or IgE to Aspergillus fumigatus
in serum*1
5. Bronchiectasis*1 *2
6. Radiologically confirmed infiltrate
7. Eosinophilia in blood (> 500/µL)
8. Precitpitating antibodies to Aspergillus fumigatus
(modifed from Agarwal [23])
*1 Obligatory criterion for defining the diagnosis.
*2 If criteria 1–4 are met but no bronchiectasis is found, the finding is
taken to be seropositive allergic bronchopulmonary aspergillosise.
underlying pathophysiology is a sensitization to Asper-
gillus fumigatus, which leads to a CD4+/TH2 mediated
inflammatory reaction. Typical symptoms include a
raised body temperature, weight loss, a drop in FEV1,
and pulmonary infiltrates on the radiograph.
Bronchiectasis can be a sequela of ABPA, but it can
also predispose to ABPA. The clinical diagnosis is diffi-
cult and is based on criteria set out by Greenberger
(overview in Agarwal [23]) (Box 2).
Acute exacerbation of ABPA usually requires treat-
ment with systemic steroids for a long period of time
(23). In order to prevent recurrence, long-term oral
therapy with itraconazole is indicated in patients with
pulmonary colonization; several studies have shown
KEY MESSAGES
● Thanks to improved antibiotic treatments and vacci-
nation programs, more congenital than postinfectious
causes of bronchiectasis have been observed.
● Different mechanisms lead to bronchiectasis, but the
pathophysiological end stage of inflammation and des-
truction is the same.
● For diagnostic purposes, a sputum specimen should be
sampled. If imaging is used then high-resolution com-
puted tomography is the method of choice.
● The treatment of the underlying disease should always
be considered. The basics of treatment include breath-
ing measures and physiotherapeutic measures. Treat-
ment of exacerbations should be based on the diag-
nostic test results for the pathogen and the antibiogram.
Addititional administration of inhaled antibiotics or
macrolides should be considered in the individual case
scenario.
● Surgical measures are available for circumscribed
bronchiectasis.
814
the effectiveness of this treatment in CF. Inhaled
amphotericin B is the subject of studies. Individual re-
ports have documented successful treatment with a
monoclonal antibody against IgE (omaluzimab).
Surgical therapy
Surgery is the method of choice in unilateral and local-
ized bronchiectasis. Several studies have shown that
resection of the bronchiectasis improved symptoms
(24). In the different studies, mortality varied from 1%
to 8.6% (e5, e6), the rate of surgical complications was
8.8–25% (e7). Complications included pneumonias,
postoperative hemorrhage, atelectasis, bronchopulmo-
nary fistula, and wound infection. In particular cases,
the resection of bilateral bronchiectasis may be the aim,
but the lesions should be limited and completely resect-
able (24). In severe complications, such as life threaten-
ing hemorrhage or fungal infection, surgical therapy
can be used as the method of last resort.
Hemoptysis, which is mostly caused by bleeds from
hypertrophied vessels of the inflamed mucosa, can be
controlled by bronchial artery embolization (coiling) if
required. This should only be done in specialized centers.
Lung transplantation in advanced disease
Lung transplantion can be a useful intervention in very
advanced non-CF bronchiectasis. It is of vital impor-
tance to identify the right time for putting the patient on
the transplant list. In accordance to the guidelines, the
following criteria should be met:
● FEV1 < 30 % and an exacerbation with inpatient
admission to intensive care, or
● More than three exacerbations per year, or
● Recurrent pneumothorax, or
● Hemoptysis requiring—and receiving—inter-
vention (25).
A double lung transplant is the method of choice in
more than 90% of cases. In case only one lung is trans-
planted, there is a risk that pathogens are transferred
from the native lung into the transplanted lung. Experi-
ences from large centers have shown that he long-term
prognosis does not differ much from that of other indi-
cations, with 5-year survival rates between 55% and
60% (e8).
Conflict of interest statement
Dr Rademacher has received honoraria for speaking from Forest and MSD.
Professor Welte has received honoraria for acting as an adviser from Novartis,
Bayer Pharma, and Gilead.
Manuscript received on 20 May 2011, revised version accepted on 27 July
2011.
Translated from the original German by Dr Birte Twisselmann.
REFERENCES
1. Bilton D: Update on non-cystic fibrosis bronchiectasis. Curr Opin
Pulm Med 2008; 14: 595–9.
2. Twiss J, Metcalfe R, Edwards E, et al.: New Zealand national inci-
dence of bronchiectasis „too high“ for a developed country. Arch
Dis Child 2005; 90: 737–40.
Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15

3. Fuschillo S, De Felice A, Balzano G: Mucosal inflammation in
idiopathic bronchiectasis: cellular and molecular mechanisms. Eur
Respir J 2008; 31: 396–406.
4. Dhillon SS, Watanakunakorn C: Lady Windermere syndrome: middle
lobe bronchiectasis and Mycobacterium avium complex infection
due to voluntary cough suppression. Clin Infect Dis 2000; 30:
572–5.
5. Fajac I, Viel M, Sublemontier S, et al.: Could a defective epithelial
sodium channel lead to bronchiectasis. Respir Res. 2008; 9: 46.
6. Patel IS, Vlahos I, Wilkinson TM, et al.: Bronchiectasis, exacerbation
indices, and inflammation in chronic obstructive pulmonary disease.
Am J Respir Crit Care Med 2004; 15; 170: 400–7.
7. Goeminne P, Dupont L: Non-cystic fibrosis bronchiectasis: diagnosis
and management in 21st century. Postgrad Med J 2010; 86:
493–501.
8. O’Donnell AE, Barker AF, Ilowite JS, et al.: Treatment of idiopathic
bronchiectasis with aerosolized recombinant human DNase I.
rhDNase Study Group.Chest 1998; 113: 1329–34.
9. Donaldson SH, Bennett WD, Zeman KL, et al.: Mucus clearance and
lung function in cystic fibrosis with hypertonic saline. N Engl J Med
2006; 354: 241–50.
10. Kellett F, Redfern J, Niven RM: Evaluation of nebulised hypertonic
saline (7%) as an adjunct to physiotherapy in patients with stable
bronchiectasis. Resp Med 2005; 99: 27–31.
11. Daviskas E, Anderson SD, Gomes K, et al.: Inhaled mannitol for the
treatment of mucociliary dysfunction in patients with bronchiecta-
sis: effect on lung function, health status and sputum. Respirology
2005; 10: 46–56.
12. Ratjen F, Munck A, Kho P, et al.: Treatment of early Pseudomonas
aeruginosa infection in patients with cystic fibrosis: the ELITE Trial.
Thorax 2010; 65: 286–91.
13. Drobnic ME, Suñé P, Montoro JB, et al.: Inhaled tobramycin in
non-cystic fibrosis patients with bronchiectasis and chronic bronchial
infection with Pseudomonas aeruginosa. Ann Pharmacother 2005;
39: 39–44.
14. Scheinberg P, Shore E: A pilot study of the safety and efficacy of
tobramycin solution for inhalation in patients with severe bronchiec-
tasis. Chest 2005; 127: 1420–6.
15. Steinfort DP, Steinfort C: Effect of long-term nebulised colistin of
lung function and quality of life in patients with chronic bronchial
sepsis. Intern Med J 2007; 37: 495–8.
Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15
MEDICINE
16. Dhar R, Anwar GA, Bourke SC, et al.: Efficacy of nebulised colomycin
in patients with non-cystic fibrosis bronchiectasis colonised with
Pseudomonas aeruginosa. Thorax 2010; 65: 553.
17. McCoy KS, Quittner AL, Oermann CM, et al.: Inhaled aztreonam ly-
sine for chronic airway Pseudomonas aeruginosa in cystic fibrosis.
Am J Respir Crit Care 2008; 178: 921–8.
18. Murray MP, Govan JR, Doherty CJ, et al.: A randomized controlled
trial of nebulized gentamicin in non-cystic fibrosis bronchiectasis.
Am J Respir Crit Care Med 2011; 183: 491–9.
19. Antoniu SA, Trofor AC: Inhaled gentamicin in non-cystic
fibrosis bronchiectasis: effects of long-term therapy. Expert Opin
Pharmacother 2011; 12: 1191–4.
20. Tsang KW, Tan KC, Ho PL, et al.: Inhaled fluticasone in bronchiecta-
sis: a 12 month study. Thorax. 2005; 60: 239–43.
21. Shinkai M, Henke MO, Rubin BK: Macrolide antibiotics as immuno-
modulatory medications: proposed mechanisms of action. Pharma-
col Ther 2008; 117: 393–405.
22. Anwar GA, Bourke SC, Afolabi G, et al.: Effects of long-term low-
dose azithromycin in patients with non-CF bronchiectasis. Respir
Med 2008; 102: 1494–6.
23. Agarwal R: Allergic bronchopulmonary aspergillosis. Chest 2009;
135: 805–26.
24. Bagheri R, Haghi SZ, Fattahi Masoum SH, et al.: Surgical manage-
ment of bronchiectasis: analysis of 277 patients. Thorac Cardiov
Surg 2009; 58: 291–4.
25. Orens JB, Estenne M, Arcasoy S, et al.: Pulmonary Scientific Coun-
cil of the International Society for Heart and Lung Transplantation.
International guidelines for the selection of lung transplant candi-
dates: 2006 update—a consensus report from the Pulmonary
Scientific Council of the International Society for Heart and Lung
Transplantation. J Heart Lung Transplant 2006; 25: 745–55.
Corresponding author:
Prof. Dr. med. Tobias Welte
Abteilung Pneumologie
Carl-Neuberg-Str. 1
30625 Hannover, Germany
welte.tobias@mh-hannover.de
@ For eReferences please refer to:
www.aerzteblatt-international.de/ref4811
815
MEDICINE

REVIEW ARTICLE

Bronchiectasis—Diagnosis and Treatment

Jessica Rademacher, Tobias Welte

eReferences
e1. Pletz MW, McGee L, Burkhardt O, et al.: Ciprofloxacin treatment
failure in a patient with resistant Streptococcus pneumoniae
infection following prior ciprofloxacin therapy. Eur J Clin Microbiol
Infect Dis. 2005; 24: 58–60.
e2. Rademacher J, Pletz MW, Welte T: Behandlung nicht mit
zystischer Fibrose assoziierter Bronchiektasen (Non-CF-
Bronchiektasen. Internist (Berl) 2010; 51: 1510–5.
e3. Bilton D, Bruinenberg P, Otulana B, et al.: Inhaled liposomal cipro-
floxacin hydrochloride significantly reduces sputum pseudomonas
aeruginosa density in CF and Non-CF bronchiectasis. Am J Respir
Crit Care Med 179; 2009: A3214.
e4. Wilson R, Welte T, Polverino E, et al.: Randomized, placebo-
controlled, double-blind, multi-center study to evaluate the safety
and efficacy of Ciprofloxacin dry powder for inhalation (Ciprofloxa-
cin DPI) compared with placebo in patients with non-cystic fibro-
sis bronchiectasis. Am J Respir Crit Care Med 183; 2011: A6407.
e5. Agasthian T, Deschamps C, Trastek VF, et al.: Surgical manage-
ment of bronchiectasis. Ann Thorac Surg 1996; 62: 976–8.
e6. Kutlay H, Cangir AK, Enön S, et al.: Surgical treatment in bron-
chiectasis: analysis of 166 patients. Eur J Cardiothorac Surg
2002; 21: 634–7.
e7. Fujimoto T, Hillejan L, Stamatis G.: Current strategy for surgical
management of bronchiectasis. Ann Thorac Surg 2001; 72:
1711–5.
e8. Weiss ES, Allen JG, Meguid RA, et al.:The impact of center vol-
ume on survival in lung transplantation: an analysis of more than
10,000 cases. Ann Thorac Surg 2009; 88: 1062–70.

8 Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 805–15

MEDICINE

BOX 2

Diagnostic criteria for allergic

bronchopulmnaory aspergillosis
1. Asthma
2. Skin reactions*1
3. Raised total IgE in serum (> 1 000 ng/L)*1
4. Raised specific IgE and/or IgE to Aspergillus fumigatus
in serum*1
5. Bronchiectasis*1 *2
6. Radiologically confirmed infiltrate
7. Eosinophilia in blood (> 500/µL)
8. Precitpitating antibodies to Aspergillus fumigatus
(modifed from Agarwal [23])
*1 Obligatory criterion for defining the diagnosis.

*2 If criteria 1–4 are met but no bronchiectasis is found, the finding is

taken to be seropositive allergic bronchopulmonary aspergillosise.

KEY MESSAGES

● Thanks to improved antibiotic treatments and vacci-

nation programs, more congenital than postinfectious
causes of bronchiectasis have been observed.
● Different mechanisms lead to bronchiectasis, but the
pathophysiological end stage of inflammation and des-
truction is the same.
● For diagnostic purposes, a sputum specimen should be
sampled. If imaging is used then high-resolution com-
puted tomography is the method of choice.
● The treatment of the underlying disease should always
be considered. The basics of treatment include breath-
ing measures and physiotherapeutic measures. Treat-
ment of exacerbations should be based on the diag-
nostic test results for the pathogen and the antibiogram.
Addititional administration of inhaled antibiotics or
macrolides should be considered in the individual case
scenario.
● Surgical measures are available for circumscribed
bronchiectasis.

6 Deutsches Ärzteblatt International | Dtsch Arztebl Int 2011; 108(48): 809–15