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                                                                                                                                                                               Case studies

                                                                                                                                                                               The authors

                                                                                                                                                                               DR PAUL KING,
                                                                                                                                                                               respiratory physician, Monash
                                                                                                                                                                               Medical Centre, Clayton, and
                                                                                                                                                                               Dandenong Hospital, Dandenong,
                                                                                                                                                                               and senior lecturer, Monash
                                                                                                                                                                               University, Victoria.

   Bronchiectasis                                                                                                                                                              ASSOCIATE PROFESSOR
                                                                                                                                                                               PETER HOLMES,
                                                                                                                                                                               respiratory physician and deputy
 Background and aetiology                                                                                                                                                      director, respiratory medicine,
                                                                                                                                                                               Monash Medical Centre,
                                                                                                                                                                               Clayton, Victoria.
BRONCHIECTASIS is defined as per-           diagnosis of bronchiectasis who were        Aetiology                                   The condition most classically associ-
manent dilation of the airways arising      subsequently found also to have             Bronchiectasis is a heterogeneous con-      ated with bronchiectasis is cystic
from chronic bronchial infection.           COPD.                                       dition and can be considered in some        fibrosis. Cystic fibrosis arises from a
   Its prevalence is unknown. There are        Several factors complicate the under-    ways to be a syndrome, like glomeru-        mutation in the cystic fibrosis trans-
reports of higher rates of bronchiectasis   standing of bronchiectasis. First,          lonephritis, rather than a discrete         membrane conductance regulator
in isolated communities (such as some       bronchiectasis blends into the general      entity. Many factors and conditions         (CFTR) gene, which is responsible for
Aboriginal communities and Alaskan          entity of chronic bronchitis, most often    are associated with it (table 1, see next   the movement of sodium and chlo-
natives) that have high rates of respi-     caused by smoking. Second, the intro-       page) but in most cases it is idiopathic.   ride by airway epithelial cells. This
ratory infection and poor health ser-       duction of antibiotics has seen a sig-      In adults bronchiectasis can be broadly     leads to tenacious sputum that cannot
vices.                                      nificant improvement in outcome,            classified as either related to or unre-    be cleared from the airways, and sec-
   Recent studies have reported that        resulting in a belief that bronchiectasis   lated to cystic fibrosis.                   ondary infection.
29-50% of patients with COPD have           is no longer a clinical problem. Finally,                                                  There are more than 1000 docu-
associated bronchiectasis on CT scan-       rather than being a single discrete         Mucociliary clearance                       mented mutations of the CFTR gene
ning, suggesting this may be a rela-        entity, bronchiectasis results from a       Mucociliary clearance is a first-line       and it has been recognised recently that
tively common condition in patients         variety of different mechanisms, with       defence against pathogenic micro-           cystic fibrosis may present atypically
with this condition. We have seen           the dominant feature being recurrent        organisms, and compromise to this           in adults with infertility (obstruction
more than 300 patients with a primary       airway infection.                           function may result in bronchiectasis.                             cont’d next page

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                                                                                                                                      20 May 2005 | Australian Doctor |      31
 How to treat – bronchiectasis

 from previous page
 of the vas deferens) and recur-
                                          Table 1: Conditions associated with bronchiectasis                Diagnosing bronchiectasis
 rent sinopulmonary infection.           Post-infectious complications
 The prevalence of undiag-               ■ Bacterial and mycobacterial infections (TB, Mycobacterium      Clinical features                  Figure 1: Sputum sample from a patient with bronchiectasis.
 nosed cystic fibrosis in adults           avium, whooping cough)                                         BEFORE the introduction of
 with bronchiectasis is not              ■ Viral (measles, adenovirus, influenza virus)                   antibiotics, patients with
 known although recent work                                                                               bronchiectasis had a poor
 suggests it is low in the Aus-          Mechanical bronchial obstruction                                 outloo, with most dying
 tralian population.                     ■ Luminal foreign body                                           before age 40. Antibiotics
    Other mucociliary defects            ■ Stenosis                                                       markedly improved outcome
 can occur in rare conditions            ■ Tumour                                                         in this condition and in the
 such as Kartagener’s syn-               ■ Lymph node                                                     1980s a major review arti-
 drome (immotile cilia) and                                                                               cle described bronchiectasis
 Young’s syndrome (mucus                 Mucociliary clearance defects                                    as an orphan disease with
 disorder). Associated factors           ■ Cystic fibrosis                                                little clinical relevance.
 that suggest there may be a             ■ Kartagener’s syndrome                                             However, since the intro-
 primary mucociliary disor-              ■ Primary ciliary dyskinesia                                     duction of high-resolution
 der are a family history of             ■ Young’s syndrome                                               CT scanning it has become
 bronchiectasis and infertil-                                                                             recognised that bronchiecta-
 ity.                                    Immune deficiency                                                sis remains a common con-
                                         ■ Hypogammaglobulinaemia                                         dition although the clinical
 Post-infectious complications           ■ IgG subclass deficiency                                        manifestations are less severe
 The aetiological factor most            ■ HIV                                                            than in the past.
 commonly associated with                                                                                    In the past 30-40 years
 bronchiectasis is childhood             Immunological over-response                                      there have been relatively
 infection. Several studies have         ■ Allergic bronchopulmonary aspergillosis                        few studies assessing the
 described bronchiectasis occur-         ■ Post-lung transplant                                           clinical features of bronchi-
 ring after childhood infections                                                                          ectasis. From the literature
 such as pneumonia, whooping             Sequelae of toxic inhalation or aspiration                       it appears the dominant
 cough and measles. However,                                                                              symptom is a chronic cough
 the following factors compli-           Rheumatic or chronic inflammatory conditions                     with purulent sputum (figure
 cate the attribution of                 ■ Rheumatoid arthritis                                           1). This symptom is present
 bronchiectasis development to           ■ Inflammatory bowel disease                                     in more than 90% of
 primary pulmonary infection:                                                                             patients and will have been
 ■ The research relies generally     and HIV infection.                                                   present for many years in
   on long-term retrospective           Bronchiectasis, which tends                                       most patients.
   recall that may result in         to be central in its distribu-                                          Patients often suffer sig-      thought of as a classic mani-
   over-ascription of bronchiec-     tion, is one of the key features                                     nificant social embarrass-         festation of bronchiectasis,        Common clinical
   tasis to infective causes.        of allergic bronchopulmonary                                         ment from their cough and          is very uncommon. There             symptoms in patients
 ■ Patients with primary disor-      aspergillosis. The latter condi-                                     many patients probably have        are usually no other abnor-         with bronchiectasis
   ders of their immune func-        tion can be caused by obstruc-                                       mild associated depression.        malities on physical exami-
                                                                                                                                                                                 ■   Chronic cough with
   tion are more likely to expe-     tion arising from fungal plugs,                                      Chest pain, which may be           nation.
                                                                                                                                                                                     purulent sputum
   rience significant and/or         an immune reaction to the                                            pleuritic, is present in 20-          In our experience most
   recurrent infections that lead    fungus in the airway wall, and                                       30% of cases.                      patients have respiratory           ■   Chronic fatigue
   to the development of             eosinophilic inflammation of                                            Haemoptysis has a similar       symptoms for more than 20
                                                                                                                                                                                 ■   Chest pain
   bronchiectasis.                   the peribronchial tissues.                                           frequency to chest pain but        years before a diagnosis of
 ■ The infections that have             It can be considered to be a                                      is a dramatic symptom and          bronchiectasis is made.             ■   Haemoptysis
   been associated with              form of uncontrolled or inap-                                        will often cause patients to       Bronchiectasis can be consid-
                                                                                                                                                                                 ■   Rhinosinusitis
   bronchiectasis most often         propriate immunological                                              seek immediate medical             ered to be a syndrome with
   (whooping cough, measles          response that results in airway                                      advice. Rarely, patients pre-      many different causes and
   and pneumonia) are                damage. Bronchiectasis can                                           sent with large-volume             these will influence the clini-    ing techniques. Recent scan-
   extremely common, with the        also occur after lung trans-                                         haemoptysis; such patients         cal features and presentation.     ners have made it possible to
   seroprevalence of whooping        plant.                                                               need immediate referral to                                            reconstruct bronchi, provid-
   cough estimated to be >50%                                                                             an emergency department.           Investigations                     ing virtual bronchoscopy.
   and that of measles >90%,         Aspiration                                                              Rhinosinusitis and fatigue      High-resolution CT scan
   in unvaccinated adults in         Aspiration may be associated                                         are very common symptoms           Bronchiectasis is diagnosed        Spirometry
   Westernised countries. The        with bronchiectasis. A higher                                        in patients with bronchiec-        using imaging techniques.          Along with asthma and
   rates of bronchiectasis           than expected incidence of                                           tasis. The incidence of rhi-       CXRs usually have non-spe-         COPD, bronchiectasis is
   would be expected to be           Helicobacter pylori sero-                                            nosinusitis may be as high as      cific findings of increased        characterised by airflow
   much higher if the causes of      prevalence has been found in                                         60-70% and ranges in sever-        lung markings, and CT scan-        obstruction, with a reduced
   bronchiectasis were this          patients with bronchiectasis.                                        ity from a mild postnasal          ning is the standard test used     FEV1, a normal or slightly
   straightforward.                  However, it has not yet been                                         drip to fulminant pansinusi-       for diagnosis.                     reduced forced vital capac-
 ■ Studies in subjects with          established that aspiration is                                       tis. In our experience most           High-resolution CT scan-        ity (FVC) and a reduced
   childhood pneumonia or            a definite cause.                                                    patients with bronchiectasis       ning using images taken in         FEV1:FVC ratio. The mech-
   whooping cough have not                                                                                will have demonstrable             1-1.5mm thicknesses shows          anism for airflow obstruc-
   demonstrated an increased         Rheumatic and chronic                                                abnormalities of their upper       the bronchi in greater detail      tion in bronchiectasis may
   incidence of bronchiectasis.      inflammatory conditions                                              airway on CT scanning.             than standard CT scanning.         arise primarily from inflam-
    However, one factor in           Another group of conditions                                             Chronic fatigue may not         When ordering a CT to              mation in the small airways.
 favour of the role of infection     associated with bronchiecta-                                         be apparent without specific       diagnose bronchiectasis it            A reduced FVC may indi-
 in initiating bronchiectasis is     sis are the chronic inflamma-                                        questioning and in our expe-       should always be specified         cate the airways are
 the high incidence of the con-      tory diseases, particularly                                          rience is present in more          as a high-resolution scan.         obstructed with mucus, have
 dition in isolated populations      rheumatoid arthritis and                                             than 70% of patients. This         The standard high-resolution       collapsed with forced exha-
 with poor health and recur-         inflammatory bowel disease.                                          may be the dominant symp-          CT criteria for diagnosing         lation or there is consolida-
 rent infection, such as Aborig-        Bronchiectasis associated                                         tom for many patients.             bronchiectasis are listed in       tion in the lung. Smoking
 inal and Polynesian peoples         with rheumatoid arthritis has                                           The effects on lifestyle        table 2 and illustrated in fig-    may be associated with
 and Alaskan Indians.                been described as preceding as                                       have not been clearly docu-        ures 3 and 4.                      worse pulmonary function.
                                     well as occurring during the                                         mented but the condition              Since the introduction of       The degree of airway
 Immune disorders                    course of the disease. In                                            does appear to reduce the          high-resolution CT it has          obstruction tends to be mod-
 An increasing number of             rheumatoid arthritis clinics the                                     activity levels of patients sig-   become much easier to diag-        erate in severity (FEV 1 of
 immune deficiencies are             incidence of bronchiectasis is                                       nificantly. Patients who are       nose bronchiectasis. In gen-       about 60% of predicted).
 clearly associated with             reported as 1-3% and several                                         working do need to take            eral it is more likely that this      Airway hyper-responsive-
 bronchiectasis. Obstructive         studies report the prevalence                                        time off to recover from           type of scanning under             ness may be associated with
 and ciliary disorders may be        of bronchiectasis on high-res-                                       exacerbations and employ-          rather than over-diagnoses         bronchiectasis. One study
 considered to be primary dis-       olution CT scans as up to                                            ers are generally not sympa-       bronchiectasis.                    found hyper-responsiveness
 orders of immune defence,           30%. The reason for this                                             thetic.                               High-resolution CT is           in 40% of patients with
 because airway clearance            association is unknown.                                                 The main finding on             becoming even more sensi-          bronchiectasis 2 and two
 mechanisms contribute an               Recurrent respiratory infec-                                      examination is the presence        tive for diagnosis, with the       other studies found 30-69%
 important component to              tions and bronchiectasis are                                         of lung crackles, which are        introduction of new tech-          of patients had a 20%
 innate and barrier immunity.        well recognised in the context                                       present in more than 60%           niques such as multi-detec-        decrease in FEV1 after hista-
 Other disorders that result in      of inflammatory bowel disease                                        of patients and are most           tor volumetric scanning,           mine challenge. 3,4 Such
 bronchiectasis       include        although the reason is also not                                      commonly bilateral and             finer cuts (0.5mm sections)        hyper-responsiveness is
 hypogammaglobulinaemia              clear.                                                               basal. Clubbing, often             and improved post-process-         probably not asthma as such

32   | Australian Doctor | 20 May 2005                                           
Figure 2: CXR of a patient with cystic bronchiectasis in the lung        Table 2: Standard                     Figure 3: Illustration of appearance of CT signs of bronchiectasis: bronchial dilatation (1),
bases.                                                                  high-resolution CT                     non tapering of bronchi (2), bronchi visible in outer 1-2cm of lung (3), bronchial thickening (4),
                                                                      criteria for diagnosing                  impacted mucus (5), crowding of bronchi (6).
                                                                     Major criteria
                                                                     ■   Abnormal widening of the
                                                                         bronchi (defined as the
                                                                         internal diameter of the
                                                                         bronchus being greater
                                                                         than the diameter of its
                                                                         adjacent pulmonary
                                                                     ■   Failure of the bronchi to
                                                                     ■   Visualisation of bronchi in
                                                                         the outer 1-2 cm of the
                                                                         lung fields
                                                                     Secondary criteria
                                                                     ■   Excessive bronchial
                                                                     ■   Impacted mucus
                                                                     ■   Crowding of the bronchi

      Table 3: Microbial isolates from patients with                                                       Table 4: Comparison of bronchiectasis, asthma and COPD
               bronchiectasis in six studies
                                                                     Variable                          Bronchiectasis                         Asthma                        COPD
 Bacteria                         Frequency (%)      Range (%)
                                                                     Cause                             Infection/immune deficiency            Airway inflammation           Smoking/inflammation
 Haemophilus influenzae           42%                29-70%
                                                                     Role of infection                 Primary                                Exacerbations                 Secondary
 Pseudomonas aeruginosa           18%                12-31%
                                                                     Sex bias                          Female                                 None                          Male
 Streptococcus pneumoniae         8%                 6-14%
                                                                     Age of presentation               40-60                                  2-20, 40-50                   60-70
 Staphylococcus aureus            7%                 5-10%
                                                                     Airway obstruction                Present                                Present                       Present
 Aspergillus fumigatus            4%                 3-7%
                                                                     Airway                            Common                                 Pathognomonic                 Infrequent
 Brahmanella catarrhalis          4%                 3-8%            hyper-responsiveness
 Other Gram-negative bacteria     2%                 0-6%            Sputum                            Purulent, copious                      Rare                          Mucoid, small volume
 Mycobacterial infections         1%                 0-5%            Signs                             Crackles                               None/wheeze                   Hyper-expansion/prolonged expiration

but occurs secondary to the                                         that H influenzae was by far            is also probably another risk          Figure 4: CT scans showing cystic bronchiectasis (top),
effects of chronic bronchial                                        the most commonly isolated              factor.                                bronchial thickening (middle) and non tapering of bronchi
inflammation.                                                       bacterium, especially in                                                       (bottom).
   For unknown reasons                                              patients during an exacerba-            Differential diagnosis
probably related to lung                                            tion.                                   As well as being part of the
pathogens, some patients                                               In adults H influenzae is            spectrum of chronic bron-
develop a rapidly progressive                                       nearly always the non-                  chitis, bronchiectasis is often
decline in respiratory func-                                        typeable form without a cap-            confused with other airway
tion. Therefore patients with                                       sule; this form is designated           diseases, particularly asthma
bronchiectasis should have                                          as non-typeable H influenzae            and COPD. The character-
spirometry performed every                                          (NTHi), in contrast to                  istic feature that distin-
2-5 years.                                                          typeable forms such as Hib.             guishes bronchiectasis from
                                                                       There are no data defin-             other lung conditions is the
Microbiology                                                        ing the role of viruses in              production of large amounts
A variety of pathogens has                                          acute exacerbations in                  of purulent sputum. In other
been isolated from the                                              patients with bronchiectasis,           ways the three conditions of
sputum of patients with                                             although viral infection has            bronchiectasis, COPD and
bronchiectasis (table 3).                                           been shown to have a role               asthma have considerable
Haemophilus influenzae is                                           in COPD. It has been shown              overlap (table 4) and some
the most frequently isolated                                        that when neutrophils from              patients clearly experience
bacterium in these studies.                                         patients with bronchiectasis            all three conditions.
When selective bacteriologi-                                        are infected in vitro with                 For many years airway
cal media and techniques are                                        strains of influenza A, lyso-           infection was thought to
used, H influenzae can be                                           some release and activity               have no role in COPD —
isolated in more than 70%                                           against bacteria are reduced.           one of the world’s most
of patients with bronchiec-                                         This may contribute to bac-             important health problems.
tasis.                                                              terial exacerbations.                   Recently there has been
   One of the problems with                                            Research has shown that              renewed interest in airway
sputum collection is that it                                        bacteria such as Psuedomonas            infection in COPD although
does not always reflect the                                         aeruginosa and H influenzae             it remains an area of contro-
flora of the airways. In one                                        stimulate neutrophilic and              versy.
study that used protected                                           inflammatory mediator release              We now know there can
bronchial brush specimens                                           in the airway. Pseudomonas              be a very high incidence of
and bronchial lavage to obtain                                      infection is associated with            bronchiectasis in COPD. A
specimens from patients with                                        more sputum, more extensive             recent landmark study found
bronchiectasis, the most com-                                       bronchiectasis on high-resolu-          the most important factor
monly isolated pathogen was                                         tion CT, more hospitalisations          associated with airway
H influenzae, found in 55%.                                         and a poorer quality of life.           obstruction in COPD was
   Another study that used                                          Pseudomonas infection gener-            infiltration of the small air-
bronchoscopic techniques to                                         ally occurs in patients with            ways with lymphocytic cells,
obtain specimens in patients                                        more severe lung disease.               suggestive of chronic bacter-
with chronic bronchitis found                                          Recurrent hospitalisation            ial infection.

                                                                                                                                              20 May 2005 | Australian Doctor |   33
 How to treat – bronchiectasis

 THE extremely high mortality rate of bronchiectasis recorded in the past           Figure 5: A: Flutter valve with a steel ball resting inside a plastic circular cone. When the patient exhales into the device
 has fallen dramatically since the introduction of antibiotics. Antibiotics         the ball moves up and down, producing oscillations of endobronchial pressure and expiratory airflow, which loosen
 remain the cornerstone of treatment for exacerbations.                             mucus to improve expectoration. B: An ‘Acapella’ device, which contains a valve that increases resistance to expiratory
   Other treatments include physiotherapy, bronchodilators, surgery,                flow to create positive pressure in the airways during exhalation.
 inhaled corticosteroids and vaccination. Most of these treatments are
 used in combination. Management and treatment plans for bronchiectasis
 are still not well defined and should be individualised.

 Antibiotic selection in bronchiectasis is complex because of the wide range
 of pathogens involved, the presence of resistant organisms and the damaged
 lung architecture. In many patients the airways are colonised simultaneously
 with multiple pathogens but in up to 50%, sputum samples show no
 growth. Some pathogens such as P aeruginosa, are protected by mucus.
    Another very important consideration is the region of the lung where the
 pathogens live. Some bacteria are predominantly extracellular (eg, S pneu-
 moniae and S aureus), some live predominantly intracellularly in bronchial
 epithelial cells and macrophages (eg, Chlamydia and Legionella) and
 H influenzae can be found extra- and intracellularly.
    Intracellular pathogens are protected from many antibiotics with poor
 cellular penetration, such as beta lactams. Giving a beta lactam with an
 antibiotic with good intracellular penetration (eg, a tetracycline, macrolide
 or quinolone) may cover a broad spectrum of pathogens.
    There is debate about whether antibiotics should only be used for exac-
 erbations or whether they should be used more regularly to control the
 chronic inflammation associated with bacterial infection. The main concern
 about the regular use of antibiotics is the development of resistance. The
 use of oral quinolones to treat Pseudomonas infections is often associated
 with resistance after 1-2 courses of treatment.
    There is some evidence to show that regular treatment with antibiotics (con-
 tinuously or on a monthly basis) produces some benefits, which include
 improved lung function and reductions in symptoms, sputum purulence and
 enzyme content, colonising microbial load in sputum, and lung inflammation.
    The route of administration is also important, with sicker patients generally
 treated intravenously. There have been two trials of nebulised antibiotics. In
 one of these, nebulised tobramycin given twice daily for one month reduced
 Pseudomonas levels in sputum but there was no change in lung function.8 In
 the other, aerosolised gentamicin administered for three days decreased
 sputum production and oxygen desaturation and improved lung function.9
    Treatment for allergic bronchopulmonary aspergillosis has revolved
 around use of systemic steroids. The addition of the antifungal agent itra-
 conazole (Sporanox) for 16 weeks in one study appeared to be significantly
 better than placebo, with benefits most marked in those who did not
 have bronchiectasis.10

 Physiotherapy and bronchopulmonary hygiene
 Optimising sputum clearance may be beneficial in patients with bronchiec-
 tasis. The benefit of physiotherapy is not limited to the clearance of
 sputum: the patient is less likely to have sputum production occurring
 unexpectedly causing social embarrassment and interrupting sleep.
    Despite use of physiotherapy for many years and several uncontrolled
 trials, a Cochrane review found little hard evidence to support chest phys-
 iotherapy and postural drainage. This is probably because it is extremely                                                    Surgery                                     that reviews patients with
 difficult to design a randomised trial for chest physiotherapy.                                                              Surgery for bronchiectasis is gen-          bronchiectasis but it is logical to
    Traditional chest percussion and tipping methods have been replaced to                                                    erally reserved for particular indi-        assume they would also benefit.
 a large extent by mechanical chest vibration or oral devices applying posi-                                                  cations, including:
 tive end-expiratory pressure. Many patients found older methods such as                                                      ■ Removing an obstructing tumour            Alternative therapies
 postural drainage uncomfortable, and newer techniques such as the use of                                                       or foreign body.                          Some patients use alterative thera-
 flutter valves and positive expiratory pressure are better tolerated (figure 5).                                             ■ Resection of an area of localised         pies such as antioxidants and
    However, it has been our experience that many patients gain significant                                                     bronchiectasis        (typically          acupuncture to help manage
 benefit from physiotherapy and that all patients should be referred to a                                                       bronchiectasis involving no more          bronchiectasis. The effects of such
 physiotherapist to be shown techniques that can be tailored to them.                                                           than one lobe).                           therapies are not known
    A review of hyperosmolar agents for bronchiectasis found that dry                                                         ■ Elimination of areas subject to

 powder mannitol improved tracheobronchial clearance, while hypertonic                                                          uncontrolled haemorrhage.                 Management of upper
 saline had not been specifically tested in bronchiectasis but improved clear-                                                ■ Removing sections of lung sus-            respiratory tract disease
 ance in normal controls and subjects with cystic fibrosis and chronic bron-                                                    pected of harbouring organisms            Many patients with bronchiectasis
 chitis.11                                                                                                                      such as M tuberculosis and                also have symptoms of chronic rhi-
                                                                                                                                M avium complex.                          nosinusitis that are treated by their
 Bronchodilators                                                                                                                 Surgical centres describe recent         GP or managed by referral to an
 A significant proportion of patients with bronchiectasis has airway                                                          results as improvement in symp-             ENT surgeon. In our experience,
 reversibility and it is logical to treat this group with bronchodilators.                                                    toms in more than 90% of                    some patients benefit from these
 (This airway reversibility is not well defined but probably arises from                                                      patients and peri-operative mor-            treatments but it is difficult to deter-
 inflamed airways rather than the permanently dilated larger bronchi.)                                                        tality of less than 3%.                     mine which patients to treat. A sub-
   Long-acting beta-2 agonists have been used for patients with bronchiec-                                                       Double lung transplantation is           jective trial of treatment is worth-
 tasis but a Cochrane review concluded there were no randomised trials to                                                     used in patients with cystic fibrosis       while if symptoms are present.
 support their use at this stage.12                                                                                           and has a 75% survival rate at one
   There are no good data to support the use of anticholinergic therapy in                                                    year and 48% at five years.                 Future treatments/immune
 bronchiectasis.                                                                                                              Patients with non-CF bronchiec-             mediators
                                                                                                                              tasis have also undergone lung              Despite aggressive treatment,
 Corticosteroids                                                                                                              transplant but survival statistics          many patients with bronchiectasis
 Oral corticosteroids have been shown to reduce morbidity in cystic fibrosis.                                                 are not available.                          have significant airway inflamma-
 It is difficult to know how much this can be ascribed to their bronchodila-                                                                                              tion in association with their
 tor effect as opposed to their anti-inflammatory properties. There are no                                                    Vaccination                                 chronic bronchial infection. This
 randomised trials to recommend the use of oral steroids in bronchiectasis.                                                   Inactivated vaccines may reduce             suggests the addition of other
    There may be some benefit from the use of inhaled corticosteroids in                                                      exacerbations in COPD, and pneu-            agents that specifically target path-
 bronchiectasis. Although decreased sputum and a trend towards improved                                                       mococcal vaccine may be useful,             ogenic micro-organisms may be
 lung function have been reported, there is still no clear evidence for their                                                 so vaccination is commonly rec-             helpful. A variety of chiefly exper-
 use. It is also possible that by decreasing host immune defence, inhaled                                                     ommended for patients with                  imental immune mediators may
 steroids could make bronchial infection worse.                                                                               COPD. There is minimal literature           become available in the future.

36   | Australian Doctor | 20 May 2005                                         
                                                                                                                                                                     1. Naidich DP, et al. Com-
Management of an                         patients whose airways are              anorexia), a past history of respira-      but there is a wide range in outcome,    puted tomography of
exacerbation in general                  colonised by multiple pathogens.        tory failure and those with resistant      with some patients having complete       bronchiectasis. Journal of
practice                               ■ Patients should be treated with         pathogens (especially Pseudomonas).        resolution of their symptoms and         Computer Assisted Tomog-
Management of an exacerbation of         antibiotics for longer than usual                                                  some having rapidly progressive dis-     raphy 1982; 6:437-44.
bronchiectasis is not well defined.      (eg, two weeks).                        Prognosis                                  ease. The factors associated with a      2. Murphy MB, et al.
Some general principles are:           ■ Referral to a chest physiotherapist     Although the outcome in bronchiec-         worse outcome are not well defined.      Atopy, immunological
■ Perform a sputum culture when-         may be beneficial.                      tasis has improved substantially, it         We have recently completed a           changes, and respiratory
  ever possible, to guide antibiotic   ■ Patients should be strongly encour-     is still a cause of excess mortality.      long-term follow-up study in             function in bronchiectasis.
  therapy.                               aged to take adequate rest and stop     The mortality rate (death arising          bronchiectasis.13 This group of 101      Thorax 1984; 39:179-84.
■ Start antibiotic therapy early.        working.                                directly from bronchiectasis) is           subjects followed for eight years had    3. Pang J, et al. Prevalence
■ Using two or more antibiotics           Patients who may benefit from          reported to be 13% over a five-year        persistent symptoms and an excess        of asthma, atopy, and
  simultaneously (eg, a beta lactam    hospitalisation or review by a respi-     follow-up period.                          loss in respiratory function compa-      bronchial hyperreactivity in
  with a macrolide or tetracycline)    ratory physician include those with          In our experience most patients         rable to that occurring in patients      bronchiectasis: a controlled
  may be helpful in the many           systemic symptoms (fevers, rigors,        continue to have ongoing problems          who smoke and have COPD.                 study. Thorax 1989;
                                                                                                                                                                     4. Bahous J, et al. Pul-
                                                                                                                                                                     monary function tests and

 Conclusion                      Authors’ case studies                                                                                                               airway responsiveness to
                                                                                                                                                                     methacholine in chronic
 BRONCHIECTASIS can be                                                                                                                                               bronchiectasis of the adult.
 considered to be a            A typical case of                                                                                                                     Bulletin Européen de Phys-
 syndrome characterised        bronchiectasis                                                                                                                        iopathologie Respiratoire
                               MRS JW, 53, presented with                                                                                                            1984; 20:375-80.
 by chronic bronchial
                               symptoms of longstanding                                                                                                              5. Angrill J, et al. Bacterial
 infection and having many
                               productive cough. She gave a                                                                                                          colonisation in patients with
 possible causes. Most
                               history of recurrent chest                                                                                                            bronchiectasis: microbiolog-
 patients have idiopathic
                               infections and sputum pro-                                                                                                            ical pattern and risk factors.
 disease. Bronchiectasis
                               duction from age eight. She                                                                                                           Thorax 2002; 57:15-19.
 can overlap with other        also complained of long-                                                                                                              6. Bandi V, et al. Non-
 respiratory conditions        standing rhinosinusitis, inter-                                                                                                       typeable Haemophilus
 such as asthma and            mittent haemoptysis and sig-                                                                                                          influenzae in the Lower Res-
 COPD.                         nificant fatigue.                                                                                                                     piratory Tract of Patients
    The main clinical             On examination she had                                                                                                             with Chronic Bronchitis.
 features are chronic          some right-sided crackles.                                                                                                            American Journal of Respi-
 productive cough,             Spirometry showed moder-                                                                                                              ratory and Critical Care
 rhinosinusitis, fatigue and   ate airway obstruction, with                                                                                                          Medicine 2001; 164:2114-
 bi-basal crackles. Most       significant improvement                                                                                                               19.
 patients have had             after bronchodilator. A high-                                                                                                         7. Hogg JC, et al. The
 symptoms for many             resolution CT scan of her                                                                                                             nature of small-airway
 years, generally from         chest showed bronchiectasis                                                                                                           obstruction in chronic
 childhood.                    in her right middle lobe and                                                                                                          obstructive pulmonary dis-
    All patients with          lingula, and a sputum                                                                                                                 ease. New England Journal
 suspected bronchiectasis      sample grew H influenzae.                                                                                                             of Medicine 2004;
 should have a high-              Mrs JW was referred to a                                                                                                           350:2645-53.
 resolution CT scan to         chest physiotherapist and                                                                                                             8. Barker AF, et al.
                               started on prn salbutamol                                                                                                             Tobramycin solution for
 establish the diagnosis.
                               and fluticasone, with regu-                                                                                                           inhalation reduces sputum
 Other important
                               lar follow-up. She was                                                                                                                Pseudomonas aeruginosa
 investigations are
                               pleased to have a clear diag-                                                                                                         density in bronchiectasis.
 spirometry and sputum
                               nosis of her disease and ben-                                                                                                         American Journal of Respi-
                               efited from regular medical                                                                                                           ratory and Critical Care
    Despite treatment,         review. Over the next five                                                                                                            Medicine 2000; 162:481-85.
 patients tend to have         years she continued to have                                                                                                           9. Lin HC, et al. Inhaled
 ongoing symptoms.             chronic symptoms and per-                                                                                                             gentamicin reduces airway
                               sistent H influenzae in her                                                                                                           neutrophil activity and
                               sputum.                                                                                                                               mucus secretion in
                                                                                                                                                                     bronchiectasis. American
                               Comment                                                                                                                               Journal of Respiratory and
                               In our experience patients                                                                                                            Critical Care Medicine
                               with bronchiectasis have long-                                                                                                        1997; 155:2024-29.
                               standing symptoms. The most                                                                                                           10. Stevens DA, et al. A ran-
                               commonly referred patients        High-resolution CT                Comment                                                           domized trial of itracona-
                               are middle-aged women, for        reveals bronchiectasis            Mild-to-moderate bronchiec-                                       zole in allergic bronchopul-
                               unknown reasons.                  with concurrent COPD              tasis will often not be appar-                                    monary aspergillosis. New
                                  Like many patients Mrs         MRS DK, 66 and an ex-             ent on a standard CT scan.                                        England Journal of Medi-
                               JW had a significant bron-        heavy smoker (45 pack-a-          When considering the diag-                                        cine 2000; 342:756-62.
                               chodilator response to salbu-     day years), presented with a      nosis of bronchiectasis, a                                        11. Wills P, Greenstone M.
                               tamol. This response may          five-year history of recurrent    high-resolution scan should                                       Inhaled hyperosmolar
                               represent asthma but is           febrile episodes and chest        always be requested.                                              agents for bronchiectasis.
                               probably more likely to           infections. She had had              The role of respiratory                                        Cochrane Database of Sys-
                               reflect airway hyper-respon-      extensive investigations and      infection in COPD remains                                         tematic Reviews
                               siveness in association with      was diagnosed as having           controversial. In our experi-                                     2001:CD002996.
                               infection.                        COPD with moderate                ence there is often consider-                                     12. Sheikh A, et al. Long-
                                  Such airway hyper-respon-      airway obstruction.               able overlap between COPD                                         acting beta-2-agonists for
                               siveness may respond to              She had remained symp-         and bronchiectasis, particu-                                      bronchiectasis. Cochrane
                               inhaled corticosteroids or        tomatic and examination           larly in patients with evi-                                       Database of Systematic
                               bronchodilators but the           showed right basal crackles.      dence of a chronic produc-                                        Reviews 2001:CD002155.
                               response is unpredictable.        A high-resolution CT scan         tive cough or recurrent chest                                     13. King PT, et al. Outcome
                               Many       patients      with     showed evidence of mild           infections.                                                       in Adult Bronchiectasis.
                               bronchiectasis have chronic       bronchiectasis in her right          Bronchiectasis should be                                       COPD. Journal of Chronic
                               non-clearing infection with       lower lobe and severe             considered in patients with a                                     Obstructive Pulmonary Dis-
                               H influenzae.                     changes of emphysema.             history of recurrent purulent                                     ease 2005; 2:27-34.
                                  Mrs JW derived signifi-           She was treated with stan-     sputum production or in
                               cant benefit from having a        dard therapy for COPD and         those with unexplained chest                                      Online resources
                               definitive diagnosis and an       bronchiectasis. Over the          crackles. The presence of                                         Australian Lung Founda-
                               explanation of her underly-       next 10 years she remained        chronic bronchial infection                                       tion:
                               ing condition. Despite med-       symptomatic and developed         in COPD may be associated                                         American Thoracic Society:
                               ical therapy she has              progressive respiratory fail-     with an accelerated decline                             
                               remained quite symptomatic.       ure.                              in respiratory function.

                                                                                                                           20 May 2005 | Australian Doctor |     37
 How to treat – bronchiectasis

     GP’s contribution
                                                                Case study                                                                                                                        and she developed anorexia                                       Patients should generally be                obtain     benefit     from
                                                                MRS VR, 72, has a 21-year                                                                                                         and right upper-quadrant                                         given at least one repeat.                  intranasal corticosteroids.
                                                                history of bronchiectasis,                                                                                                        pain. She was diagnosed                                             An inhaled corticosteroid
                                                                diagnosed by bronchoscopy                                                                                                         with gallstones, treated by                                      could be considered in all                  If more than 40% of
                                                                after episodes of dyspnoea                                                                                                        laparoscopic cholecystec-                                        patients, particularly those                patients with bronchiectasis
                                                                and infection. She has had                                                                                                        tomy. However, her                                               with airway hyper-activity.                 have airway hyper-reactiv-
                                                                bronchitis every 1-2 years                                                                                                        anorexia persisted.                                              If there is no symptomatic                  ity, is it safe to place these
                                                                despite five-yearly Pneu-                                                                                                           I have stopped her Fos-                                        improvement after 6-8                       patients on regular inhaled
        DR ASHLEY BERRY                                         movax and annual flu vac-                                                                                                         amax but her anorexia con-                                       weeks, inhaled corticos-                    corticosteroids, or does this
               Lugarno, NSW                                     cinations.                                                                                                                        tinues. Her weight has                                           teroid therapy could be                     place them at increased risk
                                                                  Seven years ago she had                                                                                                         increased to 38kg and her                                        stopped.                                    of infection and osteoporo-
                                                                a screening bone density                                                                                                          sputum is creamy rather                                                                                      sis?
                                                                scan that demonstrated                                                                                                            than purulent. She awaits                                        Mrs VR has asked about                         There is little information
                                                                osteoporosis and I started                                                                                                        gastroscopy.                                                     the Buteyko breathing                       to guide this decision. A
                                                                her on Fosamax. Mrs VR                                                                                                                                                                             method, which is gaining                    reasonable approach would
                                                                used to perform postural                                                                                                          Questions for the authors                                        acceptance in asthma.                       be to give a trial of inhaled
                                                                drainage daily but over the                                                                                                       It seems co-incidental that                                      Could this help her?                        corticosteroids for 6-8
                                                                past 12 months her compli-                                                                                                        Mrs VR had persistent                                              Some patients do seem to                  weeks to see if there is any
                                                                ance has declined.                                                                                                                pneumonia when off her B-                                        respond to such breathing                   symptomatic improvement
                                                                  Three years ago she saw a                                                                                                       group vitamins. Is there any                                     methods. Those who are                      and, if there is, use low-
                                                                naturopath, who improved                                                                                                          evidence, anecdotal or oth-                                      most likely to respond are                  dose therapy.
                                                                her diet with optimal pro-                                                                                                        erwise, of increasing immu-                                      symptomatic patients with
                                                                tein (1g/kg body weight),                                                                                                         nity with diet and/or vita-                                      reasonable lung function.                   Is it safe to use nebulised
                                                                increased amounts of veg-                                                                                                         mins, thereby reducing                                                                                       gentamicin as prophylaxis
                                                                etables and fruit and a pow-                                                                                                      infective episodes?                                              General questions for the                   against infections through
                                                                dered multi-vitamin (mainly                                                                                                          There is no clear evidence                                    authors                                     winter?
                                                                B-group vitamins) multi-                                                                                                          of any benefit.                                                  Given that recurrent rhino-                   Theoretically yes, but
                                                                mineral supplement.                                                                                                                                                                                sinusitis occurs in more                    inhaled gentamicin has not
                                                                  Co-incidentally, since                                                                                                          Mrs VR drains 0.5-1 cup of                                       than 70% of patients with                   been used in this context.
                                                                then she remained infection                                                                                                       creamy sputum daily. If it                                       bronchiectasis, is it worth
                                                                free until December 2004,                                                                                                         becomes green or if there is                                     doing skin testing for                      Are the flutter valve and
                                                                when she required repeated                                                                                                        haemoptysis or systemic                                          inhaled allergens and plac-                 Acapella devices (to increase
                                                                hospitalisation for persistent                                                                                                    symptoms, I prescribe                                            ing all patients on inhaled                 expiratory airways pressure)
                                                                pneumonia. Interestingly,                                                                                                         Rulide. Is this adequate?                                        intranasal corticosteroids?                 readily available and what
                                                                she had run out of her vita-                                                                                                      When should I consider an                                           As rhinosinusitis seems to               do they cost?
                                                                min some months earlier.                                                                                                          inhaled corticosteroid?                                          be infective rather than                      Flutter     valves     and
                                                                  Mrs VR was admitted                                                                                                                Rulide is a good first-                                       allergic in origin, testing for             Acapella devices are readily
                                                                three times before she                                                                                                            choice antibiotic because of                                     inhaled allergens is gener-                 obtained from physiothera-
                                                                improved. Her weight                                                                                                              its broad spectrum of action                                     ally not useful. In our expe-               pists at a cost of about
                                                                dropped from 42kg to 35kg                                                                                                         and low risk of side effects.                                    rience, patients rarely                     $100.

                                                         How To Treat Quiz                                                                                                                        Complete this quiz to earn 2 CPD points and/or 2 PDP points by marking the correct answer(s)
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     1. Which ONE statement about                                                                 ❏    a) Haemoptysis                                                                             6. Which TWO signs would you expect to                                                      9. To optimise sputum clearance Norma is
     bronchiectasis is correct?                                                                   ❏    b) Chest pain                                                                              find when examining Norma if she does                                                       referred to a physiotherapist. Which TWO
     ❏ a) Airway hyper-responsiveness is never                                                    ❏    c) Fatigue                                                                                 have bronchiectasis?                                                                        newer treatments might the
        associated with bronchiectasis                                                            ❏    d) Weight loss                                                                             ❏ a) Pleural effusion                                                                       physiotherapist use?
     ❏ b) Bronchiectasis is a temporary dilation                                                                                                                                                  ❏ b) Clubbing                                                                               ❏ a) A flutter valve
       of the airways caused by chronic                                                           4. Tony relates his symptoms to a                                                               ❏ c) Lung crackles                                                                          ❏ b) Teaching Norma’s partner to percuss
       bronchial infection                                                                        childhood infection. Which THREE                                                                ❏ d) Evidence of rhinosinusitis                                                               the chest
     ❏ c) Bronchiectasis may be associated                                                        infections are most commonly associated                                                                                                                                                     ❏ c) Postural drainage
       with COPD                                                                                  with bronchiectasis?                                                                            7. Which ONE investigation is most useful                                                   ❏ d) A positive expiratory pressure device
     ❏ d) The five-year mortality rate (directly                                                  ❏ a) Pertussis                                                                                  for confirming the diagnosis of
       related to bronchiectasis) is 4%                                                           ❏ b) Measles                                                                                    bronchiectasis in Norma?                                                                    10. Some months after diagnosis Norma
                                                                                                  ❏ c) Pneumonia                                                                                  ❏ a) Sputum culture                                                                         presents with an increased production of
     2. Which ONE condition is not associated                                                     ❏ d) Varicella                                                                                  ❏ b) High-resolution CT scan of the chest                                                   purulent sputum and lethargy. Clinical
     with bronchiectasis?                                                                                                                                                                         ❏ c) Spirometry                                                                             signs are unchanged and she is afebrile. A
     ❏ a) Rheumatoid arthritis                                                                    5. Norma, 65, has had a chronic cough for                                                       ❏ d) Chest X-ray                                                                            sputum culture is arranged but no
     ❏ b) Hypogammaglobulinaemia                                                                  five years and you are considering                                                                                                                                                          organism is isolated. Which TWO antibiotic
     ❏ c) Sarcoidosis                                                                             bronchiectasis in the differential diagnosis.                                                   8. Bronchiectasis is confirmed. Which                                                       regimens might you use?
     ❏ d) Cystic fibrosis                                                                         Which ONE feature distinguishes                                                                 pathogen is the ONE most likely to be                                                       ❏ a) Tetracycline and a beta lactam for two
                                                                                                  bronchiectasis from other lung conditions?                                                      isolated from Norma’s sputum?                                                                 weeks
     3. Tony, 35, is a non-smoker who has a                                                       ❏ a) Hyperexpansion                                                                             ❏ a) Pseudomonas aeruginosa                                                                 ❏ b) Penicillin for three weeks
     chronic cough with purulent sputum.                                                          ❏ b) Wheeze                                                                                     ❏ b) Aspergillus fumigatus                                                                  ❏ c) Aquinolone for three weeks
     Which ONE other symptom does not                                                             ❏ c) Airways obstruction                                                                        ❏ c) Haemophilus influenzae                                                                 ❏ d) A macrolide and a beta lactam for two
     commonly occur in a patient with                                                             ❏ d) Copious purulent sputum                                                                    ❏ d) Streptococcus pneumoniae                                                                 weeks


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     NEXT WEEK The next How To Treat focuses on alcohol problems. The author is Associate Professor Katherine M Conigrave, staff specialist and associate professor in medicine, psychological medicine
     and public health, University of Sydney, in collaboration with the other authors of the Drink-less Program, school of public health, University of Sydney.

38     | Australian Doctor | 20 May 2005                                                                                                                    

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