doi:10.1510/icvts.2011.

284208

Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625

www.icvts.org

State-of-the-art - Pulmonary
Non-cystic fibrosis bronchiectasis
Paulo C. Neves*, Miguel Guerra, Paulo Ponce, José Miranda, Luís Vouga
Department of Cardiothoracic Surgery, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal

Received 18 July 2011; accepted 5 August 2011

Summary

Bronchiectasis is characterized by irreversible widening of the medium-sized airways, with inflammation, chronic bacterial infection and
destruction of the bronchial walls. Exercise or inspiratory muscle training may improve quality of life and exercise endurance in people with
non-cystic fibrosis bronchiectasis. Prolonged-use antibiotics improve clinical response rates, but may not reduce exacerbation rates or lung
function. Surgery is often considered for people with extreme damage to one or two lobes of the lung who are at risk for severe infection
or bleeding. In this review, the authors will focus on non-cystic fibrosis bronchiectasis, pointing out the differences in management when
compared with the cystic fibrosis context, with special emphasis on surgical management.
 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

Keywords: Bronchiectasis; Fibrosis; Lung; Non-cystic; Transplantation

1. Introduction features are an internal diameter of a bronchus wider than

Bronchiectasis was described by Laennec [1] in 1962 as
an affection of the bronchia, which ‘is always produced
by chronic catarrh, or by some other disease attended by
long, violent and often repeated fits of coughing’. It is a
pathological description of a disease process that has many
possible causes. The characteristic features are abnor-
mally dilated, thick-walled bronchi that are inflamed and
its adjacent pulmonary artery, a failure of the bronchi to
taper and the visualization of bronchi in the outer 1–2 cm
of the lung fields [3].
With the widespread availability of HR-CT, it has been con-
cluded that bronchiectasis remains a common and impor-
tant cause of respiratory disease. It has been estimated
that there are at least 110,000 adults in the USA with this
condition [4]. Besides this, there is an overlap with chronic

chronically infected by bacteria. It is a chronic condition,
which can be a significant physical problem, causing social
morbidity.
Non-cystic fibrosis bronchiectasis (NCFB) can be catego-
rized according to the pathological or radiographic appear-
ance of the airways (Fig. 1):
•• Cylindrical or tubular bronchiectasis is characterized by
dilated airways alone and is sometimes seen as a resid-
ual effect of pneumonia.
•• Varicose bronchiectasis (with an appearance similar to
that of varicose veins) is characterized by focal con-
strictive areas between the dilated airways caused by
defects in the bronchial wall.
•• Saccular or cystic bronchiectasis is characterized by
progressive dilatation of the airways, which end in large
cysts, saccules or grape-like clusters (this being the
most severe form of bronchiectasis) [2].
NCFB is currently, diagnosed using high-resolution com-
puted tomography (HR-CT) scanning. The main diagnostic
*Corresponding author. Serviço de Cirurgia Cardiotorácica, Centro Hospitalar
de Vila Nova de Gaia/Espinho, EPE, Rua Conceição Fernandes, s/n 4434-502
Vila Nova de Gaia, Portugal. Tel.: +351-227865100; fax: +351-227865170.
E-mail address: pauloacn@gmail.com (P.C. Neves).
 2011 Published by European Association for Cardio-Thoracic Surgery
State-of-the-art
obstructive pulmonary disease (COPD), with a reported
incidence of bronchiectasis in COPD of between 29% and
50% [5]. There are also reports [6] of a high prevalence in
relatively isolated populations with poor access to health
care and high rates of respiratory tract infection during
childhood.
2. Etiology
The most common cause of NCFB in the literature is
post-infectious. This is not a well-defined entity, but the
usual context appears to be one acute infectious episode
that is thought to result in bronchiectasis. A possible
mechanism for post-infectious NCFB is a significant infec-
tion in early childhood which causes structural damage to
the developing lung, allowing perpetual bacterial infec-
tion. Over time, persistent infection may then result in
bronchiectasis.
Muco-ciliary clearance is a key defense mechanism against
pulmonary infection. Recently, there has been renewed
interest in ciliary defects [7]. The main ciliary disorder is
primary ciliary dyskinesia, which combines upper and lower
respiratory tract infection with NCFB formation, male infer-
tility and, in approximately 50% of cases, situs inversus.
620 P.C. Neves et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625
Fig. 1. The three different types of bronchiectasis. Cylindrical or tubular
bronchiectasis involves dilated airways alone. Varicose bronchiectasis
is characterized by focal constrictive areas between the dilated airways.
Saccular or cystic bronchiectasis is characterized by progressive dilatation
of the airways, which form grape-like clusters. (Adapted from www.
radiopaedia.com.)
Patients with humoral immunodeficiency syndromes involv-
ing deficiencies of IgG, IgM, and IgA are at risk for recurrent
suppurative sinopulmonary infections and NCFB [8].
NCFB is also associated with rheumatoid arthritis (1–3%)
[9]. In this context, it has been described as preceding
the arthritis, as well as occurring during the course of the
disease.
Repeated respiratory tract infections and bronchiecta-
sis have been noted in patients with inflammatory bowel
disease, most often in those with chronic ulcerative coli-
tis [10]. Postulated mechanisms include the infiltration of
the airway by immune effector cells, such as lymphocytes,
enhanced autoimmune activity as part of the underlying
disease, and complications of immune-modulating thera-
pies. Bowel resection does not improve the respiratory
symptoms and may even worsen the progression of the
disease.
Patel et al. [5] found that 50% of patients with COPD had
co-existent bronchiectasis, and this was associated with
bacterial airway colonization, inflammatory markers and a
longer duration of exacerbations. Patients with COPD and
NCFB tend to have more dyspnea, worse lung function and a
lack of upper airway involvement when compared to other
subjects with bronchiectasis [11]. There is also some over-
lap in the pathology of COPD and NCFB. Both conditions
have neutrophils and T-lymphocytes as the predominant
inflammatory cell, pulmonary damage caused by protease
release and airflow obstruction worsened by lymphoid fol-
licles [12].
Beyond these etiological factors, there are many others
that correlate with NCFB, such as extremes of age, mal-
nutrition, socioeconomic disadvantage and α-1-antitrypsin
deficiency.
3. Pathophysiology and distribution
NCFB is characterized by mild-to-moderate airflow
obstruction that generally worsens over time. The most
widely known model of the development of bronchiecta-
sis is Cole's ‘vicious cycle hypothesis’ [13]. This proposes
that an environmental insult, often against a background
of genetic susceptibility, impairs mucociliary clearance,
resulting in persistence of microorganisms in the bronchial
tree and microbial colonization. The microbial infection
causes chronic inflammation, resulting in tissue damage
and impaired mucociliary motility. This then leads to more
infection, with a cycle of progressive inflammation, causing
lung damage. The current viewpoint requires two factors
for the development of this condition: persistent infection
and a defect in host defenses.
Although there are no studies of patients in the very early
stages of bronchiectasis, findings in patients with proven
NCFB give weight to the importance of enhanced cellular
and mediator responses: bronchial mucosal biopsies reveal
infiltration by neutrophils and T-lymphocytes. An analysis
of expectorated sputum shows increased concentrations
of elastase and the chemoattractants interleukin-8, tumor
necrosis factor-α and prostanoids [2].
The distribution of NCFB may be associated with different
pathophysiological processes. Allergic bronchopulmonary
aspergillosis is classically associated with central bronchi-
ectasis. NCFB has been described as being localized (i.e.
confined to one lobe) or generalized. Most commonly, this
condition is generalized, and seems to be usually found
in the lower lobes [14]. The involvement of the lower
lobes may reflect gravity-dependent retention of infected
secretions.
Three types of focal airway obstruction may lead to NCFB.
One type is luminal blockage by a foreign body, broncholith
or slowly growing tumor that is usually benign.
A second type of obstruction is extrinsic narrowing due to
enlarged lymph nodes. One good example is middle lobe
syndrome, which involves a small angulated orifice sur-
rounded by a collar of lymph nodes that may enlarge and
encroach on the main airway after infection with granulo-
matous disease-causing organisms, such as mycobacteria or
fungi [15].
A third type of obstruction is twisting or displacement
of the airways after a lobar resection (i.e. the occasional
apical displacement of a lower lobe after surgery for the
resection of the upper lobe). Recurrent lobar pneumonia is
a distinguishing feature of the first two types of focal NCFB
and is important to recognize, since interventional bron-
choscopy or surgery may offer palliation and sometimes
cure.
4. Symptoms
The symptoms and clinical course of NCFB are variable.
Some patients have no symptoms at all or symptoms only
during exacerbations, whereas others have them daily.
The classic clinical manifestations of this clinical condi-
tion are cough and daily mucopurulent sputum produc-
tion, often lasting months to years. Blood-streaked sputum
or hemoptysis may result from airway damage associated
 P.C. Neves et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625 621

with acute infection. Dyspnea and wheezing occur in 75%

of patients. Pleuritic chest pain occurs in 50% of patients
and reflects the presence of distended peripheral airways
or distal pneumonitis adjacent to a visceral pleural surface.
Physical examination of the chest shows adventitious breath
sounds, such as crackles (70%), wheezing (34%) or rhonchi
(44%) [2].
A number of patients have frequent exacerbations of
their symptoms due to NCFB. Exacerbations can be defined
as patients reporting four or more of the following symp-
toms: change in sputum production, increased dyspnea,
increased cough, fever over 38 °C, increased wheezing,
decreased exercise tolerance, fatigue, malaise, lethargy,
reduced pulmonary function, changes in chest sounds or
radiographic changes consistent with a new infectious
process [16].

5. Diagnosis

Bronchography-proven bronchiectasis shows that chest Fig. 3. Computed tomography scan of a patient with non-cystic fibrosis
radiography is normal in only 7.1% of patients. Common bronchiectasis localized to the lower lobes. A multicystic space within
findings in this setting include crowding of the bronchi, loss the base of the lower lobes is continuous with the small airways and is
of definition of the bronchovascular markings, oligemia and, representative of cystic bronchiectasis. Thick-walled circular structures
called ‘signet rings’ can be seen. (Courtesy of Centro Hospitalar de Vila Nova
in more severe disease, honeycombing, air–fluid levels or de Gaia/Espinho.)
fluid-filled nodules (Fig. 2). Therefore, careful examination
of chest radiographs is essential [17]. When CT appeared
as a diagnostic tool, it replaced bronchography as the gold
standard for the diagnosis of NCFB.
Spirometry often shows a limitation of airflow, with a
Cylindrical malformation appears as ‘tramlines’ or, when
reduced ratio of forced expiratory volume in one second
imaged in cross-section, as thick-walled circular structures
(FEV1) to forced vital capacity (FVC), a normal or slightly
called ‘signet rings’ (Fig. 3). Varicose bronchiectasis has a
reduced FVC, and a reduced FEV1. A reduced FVC may indi-
typical beaded appearance, and saccular or cystic changes
cate that airways are blocked by mucus, that they collapse
appear as a string of cysts, a cluster of grapes, or even as
with forced exhalation, or that there is pneumonitis in the
air–fluid levels due to retained secretions.
lung [18].
Currently, HR-CT is the best tool for diagnosing NCFB, clar-
Diagnosing NCFB on radiological imaging needs further diag-
ifying the findings from chest radiography and standard CT,
nostic work-up to reveal the underlying cause. HR-CT-scans do

State-of-the-art
and mapping airway abnormalities that cannot be seen on
not contribute towards identifying the etiology and, most
plain films of the chest.
importantly, a specific etiological diagnosis frequently leads
to a change in management. Other tests may indicate the
underlying etiology, including a detailed patient history,
biochemical evaluation of a venous blood sample, radiology,
pulmonary function tests, sputum investigations and ciliary
function tests [19].

6. Management

6.1. Physiotherapy

The treatment aims in adult care are to control the symp-

Fig. 2. Plain radiograph of a patient with severe unilateral non-cystic fibrosis
bronchiectasis showing severe cystically dilated bronchi most marked in the
left lung and caused by tuberculosis. This radiograph also reveals crowding of
the bronchi, loss of definition of the bronchovascular markings and oligemia.
(Courtesy of Centro Hospitalar de Vila Nova de Gaia/Espinho).
toms and thus enhance quality of life, reduce exacerbations
and maintain pulmonary function [20]. It is important that
treatments are carefully evaluated and, in particular, that
treatments routinely used in cystic fibrosis are not simply
translated into use in NCFB [21].
The increased production of mucus, together with impair-
ment of the mucociliary system, leads to mucus accumula-
tion, cough and recurrent infections. Patients with NCFB
may benefit from interventions to help clear the excessive
secretions. Physiotherapy is considered one of standard
treatments when dealing with cystic fibrosis, but evidence
for its use in the context of non-cystic fibrosis is limited
622 P.C. Neves et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625

[22]. This comprises postural drainage and techniques, such fibrosis bronchiectasis, treatment with aerosolized anti-
as the active cycle breathing technique. pseudomonal antibiotics is recommended [33].

6.2. Vaccination 6.5. Acute exacerbations

There are no randomized controlled trials determin- Antibiotics are recommended for exacerbations that pres-
ing the effectiveness of influenza vaccination in patients ent with acute deterioration (usually over several days)
with NCFB. There is limited evidence to support the use with worsening local symptoms (cough, increased sputum
of 23-valent pneumococcal vaccination as routine manage- volume or change of viscosity, increased sputum puru-
ment in adults and children with NCFB [23]. lence with or without increasing wheeze, breathlessness,
and hemoptysis) and/or systemic upset (Fig. 4). The drug
6.3. Airway pharmacotherapy selected should be active against Haemophilus influenzae
and Staphylococcus aureus. For patients with more severe
Most of the agents that have been used attack the physi- disease, recent antibiotic treatment or cystic fibrosis, an
cal properties of the mucus. Hyperosmolar inhalation anti-pseudomonal treatment should also be considered
has been shown to improve airway clearance in all the [34]. Sputum culture should be performed with sensitivity
major chronic diseases characterized by sputum reten- testing in order to guide further therapy.
tion. Hypertonic saline may provide an useful adjunct to Bronchodilator therapy can be added in the case of obstruc-
physiotherapy [24]. tion or symptoms, as well as steroid therapy, although there
The potential for recombinant human DNase to favorably are no randomized controlled trials on this. High doses of
influence symptoms in NCFB has not been tested in children bromhexine can facilitate sputum expectoration [25].
[25], but it has been well studied in adults and there is
no evidence of benefit [26]. The Cochrane database sug- 6.6. Surgery
gests that bromhexine is the only mucolytic so far shown
to be beneficial in the treatment of NCFB exacerbations There are studies [35] reporting lung resection surgery
[25]. No randomized controlled trials have investigated the for bronchiectasis in both children and adults, aiming to
role of short- and long-acting bronchodilators in NCFB [27]. improve the symptoms. The indications for surgery are
Some adults may show a favorable response with the use listed in Table 1.
of anticholinergic drugs, such as ipratropium bromide [28]. The goals of surgical therapy for NCFB are to improve
Methylxanthines, such as theophylline and aminophylline the quality of life for those patients in whom medical
are not useful in the treatment of NCFB [29]. Current evi- treatment has failed and to solve complications, such
dence does not support routine use of inhaled corticoste- as empyema, severe or recurrent hemoptysis, and lung
roids in adults with NCFB [30]. abscess [35]. Complete and anatomical resection should
be done with preservation of as much lung function as
6.4. Antimicrobial therapy possible in order to avoid cardiorespiratory restriction.
There are important factors to consider in the selection of
Antimicrobial therapy, mostly antibiotics, has been tried patients, such as their respiratory function and performance
systemically and via inhalation. When systemic antibiotics
are used, the strategies applied differ greatly. Sometimes
they are used as needed or schematically, alternating one
or more antibiotics.
Overall, chronic antibiotic treatment is currently, not rec-
ommended, but there is one exception: macrolide therapy.
This class of antibiotic has a non-antibiotic effect on bron-
chiectatic inflammation. Total bronchoalveolar lavage cell
count, sputum volume, interleukin-8 level and neutrophil
level all seem to decrease with macrolide use. Patients with
frequent exacerbations of NCFB who had been treated with
rescue antibiotics showed an improvement in exacerba-
tion frequency, spirometry and sputum microbiology when
treated with long-term low-dose azithromycin [31]. This
explains the current widespread use of macrolide therapy
for bronchiectatic patients.
Inhaled antibiotics have more limited side effects.
However, they are more expensive and may be less well
tolerated because of bronchospasm. Nebulized tobramy-
cin decreased the number of admissions and days in hospi-
tal. Some studies [32] showed a possible positive effect of
Fig. 4. Definition of an exacerbation needing antibiotic therapy. Antibiotics
prolonged (more than four weeks) antibiotic use for NCFB are recommended in non-cystic fibrosis bronchiectasis for exacerbations that
in selected patients. Overall, antibiotics are rarely used present with an acute deterioration with worsening local symptoms and/or
in clinical practice for NCFB, but in patients with cystic systemic rebound of the disease. (Adapted from Pasteur et al. [21].)
 P.C. Neves et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625
Table 1.  Indications for surgery in bronchiectasis
Failure of medical/conservative therapy
Recurrent respiratory infections
Persistent sputum production
Hemoptysis
Chronic cough
Persistent lung abscess
Adapted from Pasteur et al. [21].
status, which must be compatible with the anesthetic risk.
In addition, the resection should be performed early in the
natural history of the disease because of the risk of the
contamination of healthy bronchi [35].
Surgery should be delayed in cases of severe inflamma-
tion until adequate control has been achieved. In addition,
the preoperative treatment should include reducing air-
way obstruction and eliminating microorganisms from the
lower respiratory tract, therefore consisting of antimicro-
bial therapy, postural physiotherapy, bronchodilators and
corticosteroids.
The presence of multiple or bilateral NCFB is generally con-
sidered to be a contraindication to operation [36]. Surgery
for bilateral NCFB is considered risky, and hemorrhagic or
infectious complication are expected because of the bron-
chial arterial circulation and septic context. However, some
groups [37] have proposed radical operations for bilateral
NCFB, although others have reported higher operative
mortality [36]. It seems that limited operations should be
offered to some patients with diffuse NCFB.
In this context, patients who may benefit from surgery
have the following indications: (1) disease that is symp-
tomatic, is unresponsive to medical treatment and can be
completely resected; (2) hemoptysis that cannot be con-
trolled or recurs after bronchial artery embolization; and
(3) a need for palliative surgery, in which the most involved
lobe or segments are resected to improve the symptoms.
In select individuals, limited resections of targeted seg-
ments may achieve longstanding symptomatic improvement.
Maziéres et al. [36] observed that a total disappearance
or regression of preoperative symptoms occurred in 75% of
such patients. In a recent study [38], the authors suggest
that surgery for bilateral NCFB can be performed with mor-
bidity and mortality patterns quite similar to those results
in localized disease, with a disappearance or regression of
preoperative symptoms in 83% of patients.
Eren et al. [39] reported postoperative complications in
11% of patients who underwent complete resection and
in 80% of those who underwent incomplete resection. The
same authors stated that, beyond incomplete resection, a
history of tuberculosis was a risk factor both for postop-
erative complications and for a worse operative result. The
lack of a preoperative bronchoscopic examination and a low
FEV1 were risk factors for postoperative complications.
A recent retrospective analysis [40] showed that advanced
age (P=0.04) and renal failure (P=0.001) were related to
postoperative mortality, and that preoperative renal failure
was associated with mortality (P=0.025). The same study
reported that, after surgery, 478 (60.5%) patients were
asymptomatic, 111 (14.1%) had improved, and 117 (14.8%)
showed no improvement or a worsened condition.
623
Prieto et al. [41] reported that, in a series of 119 patients,
73 patients (68%) were asymptomatic after surgery, and 31
(29%) showed significant clinical improvement, whereas
only four (3.7%) maintained or worsened their prior symp-
toms, after a mean follow-up of 4.5 years. The same authors
concluded that the best clinical improvement occurred in
patients with complete resection of the disease (P=0.008).
A study performed in our institution showed no mortality
and an overall morbidity of 15.7%, with a mean follow-up
of 3.4 years [42].
When suggestive lung regions are not excised, with the aim
of sparing as much lung tissue as possible, more postopera-
tive complications occur and a second operation that car-
ries a higher morbidity and mortality might be required to
remove the residual diseased tissues [43]. Therefore, during
intraoperative examinations, if suggestive areas that could
not be determined by radiological examination are pres-
ent, these parenchymal areas should be resected in order to
perform complete resection and to decrease relapse rates
[39]. Indirect signs that may be helpful during operation
are poor expansion of the involved segments or lobes, the
actual palpation of ectatic bronchi, and a lack of anthra-
cotic pigment over the abnormal lung, indicating a lack
of function. If there are affected areas in different lobes,
multiple-segment resection can be performed in order to
protect as much pulmonary function as possible [44].
Technically, surgery for NCFB can be either very easy or
extremely difficult. A double-lumen tube should always be used
to avoid possible intraoperative contamination of the contra-
lateral lobes. The surgeon may experience some technical dif-
ficulties caused by dense adhesions, hyperplastic lymph nodes
around the pulmonary artery and its branches, and incomplete
fissures. Because the bronchial arteries are usually hypertro-
phied, special care should be taken with these vessels in order
to avoid postoperative bleeding. Besides technical issues, as
well as pre-operative pleuropulmonary infection, the incidence
State-of-the-art
of postoperative bronchopleural fistula after lung resection for
NCFB is identical with surgery for lung cancer.
Surgery can be performed via thoracotomy or can be
video-assisted. A recent study [45] showed that video-
assisted thoracoscopic lobectomy in localized NCFB is a safe
and more efficient procedure for selected patients, with
better recovery.
6.7. Lung transplantation
Lung transplantation is available for end-stage cardiopul-
monary disease in children and adults, although there is
a lack of literature specifically relating to bronchiectasis.
In fact, specific guidelines for the referral of patients with
NCFB have not been established. Therefore, parameters
similar to those set for cystic fibrosis patients can eventu-
ally be applied to patients with NCFB [46].
The indicators of end-stage lung disease in bronchiectasis
are listed in Table 2. In general, patients should be consid-
ered for transplantation if there is <a 50% probability of
survival for two years without transplantation, quality of
life is likely to be improved by transplantation, there are no
contraindications to transplantation, and patients are fully
informed of the risks and benefits of lung transplant and
fully committed to proceeding with evaluation and potential
624 P.C. Neves et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 619-625
Table 2.  Indicators of end-stage lung disease in bronchiectasis
Postbronchodilator FEV1 <30% (especially young females with cystic fibrosis)
Exacerbation of pulmonary disease requiring intensive care unit admission
Increasing frequency of exacerbations requiring intravenous antibiotics
Refractory and/or recurrent pneumothorax
Recurrent hemoptysis not controlled by embolization
Other factors to consider:
  Hypercarbia (PaCO2 >45 mmHg)
  Rapidly progressive decline in lung function
  Resting hypoxemia (PaO2 <55 mmHg)
FEV1, forced expiratory volume in one second; PaCO2, partial pressure of
carbon dioxide in arterial blood; PaO2, partial pressure of oxygen in arterial
blood. Adapted from Hayes and Meyer [46].
listing. Referred patients should have a rigorous evaluation
to determine whether they meet criteria for being placed
on the waiting list and to identify co-morbidities that can
complicate further management.
Because the number of lung transplants performed for
patients with NCFB is much smaller than that for patients
with cystic fibrosis, few data are available concerning patient
selection, choice of procedure and outcomes. Nonetheless,
bilateral lung transplantation is widely accepted in this
patient population, with the assumption that infection in
the contralateral native bronchiectatic lung would threaten
single-lung transplantation [47].
The current medical literature includes only one study that
has examined lung transplantation for NCFB [48]. This study
included 22 patients (12 males, 10 females) with an average
age of 43.4 years (range 20–58) years who underwent lung
transplantation for this condition over a 13-year period.
Bilateral sequential lung transplants were performed in four
patients, double-lung transplants with tracheal anastomosis
in five, heart–lung transplants in six, and single-lung trans-
plantation in seven. A total of five out of seven patients who
underwent a single-lung transplant had minimal sputum
production, whereas four out of seven had asymmetrical
findings on a CT-scan of the chest. The authors therefore,
concluded that a good outcome is possible after single-lung
transplantation in selected patients [49]. Survival and lung
function after transplantation for NCFB were similar to
those after transplantation for cystic fibrosis.
7. Prognosis
Keistinen et al. [50] reviewed the National Hospital
Discharge Register in Finland and identified 842 patients
with bronchiectasis. During a follow-up period of between
8.0 and 12.9 years, the number of hospitalizations for
patients with bronchiectasis varied widely (range 1–51,
mean 2.2). Mortality was 28% among patients with bronchi-
ectasis, 20% among asthma patients and 38% among COPD
patients.
Onen et al. [49] showed that, in NCFB, factors associated
with higher mortality were advanced age, poor functional
status, more severe disease based on radiographic findings,
and evidence of hypoxemia or hypercapnia.
Bronchiectasis associated with cystic fibrosis carries a
worse prognosis than NCFB. Current mortality is difficult to
estimate, given the difficulty in identifying the prevalence
and the lack of definitive studies. Overall, the prognosis
for bronchiectasis varies with the underlying or predispos-
ing condition.
8. Conclusions
The introduction of HR-CT has improved the diagnosis
of bronchiectasis, and emphasis should now be placed on
the role of treatment and follow-up measures, as well as
the influence of concomitant diseases, as the prognosis for
these patients is poorer than that for asthmatics.
Medical management has improved in recent years,
improving the prognosis of NCFB. However, there are few
randomized controlled trials supporting evidence for the
management of NCFB, which has to be clearly distinct
from that of cystic fibrosis because of the differences in
pathophysiology.
With regard to surgical management, it is clear that sur-
gery for NCFB can nowadays be performed with minimal
complications and that it improves symptoms and quality
of life, mostly in younger patients with localized disease.
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