Non-cystic fibrosis bronchiectasis by ProQuest


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									CME Respiratory medicine

Non-cystic fibrosis bronchiectasis                                                                   tomography of the chest. The defining
                                                                                                     characteristic is bronchial dilatation with
                                                                                                     the internal diameter of the bronchial
                                                  Pathologically, there is abnormal perma-           lumen greater than that of the adjacent
Maeve P Murray MB ChB MRCP, Clinical
                                                  nent dilatation of the airways. This leads         artery, categorised as tubular, varicose or
Research Fellow
                                                  to impaired mucociliary clearance, which           cystic (Figs 1(a), 1(b) and 1(c)).5
Adam T Hill MB ChB MD FRCPE, Consultant
Respiratory Physician and Honorary Senior         in turn leads to a vicious cycle of bacterial
Lecturer                                          colonisation in normally sterile airways           Aetiology
Department of Respiratory Medicine, Royal
                                                  and excessive bronchial inflammation.
                                                  This review explores current clinical              No underlying cause is identified in up to
Infirmary of Edinburgh, Scotland
                                                  practice for this complex condition.               50% of cases and is post-infective in up
Clin Med 2009;9:164–9
                                                                                                     to 42%.6,7 The common causes, appro-
                                                                                                     priate investigations and expected
                                                  Diagnosis                                          abnormal findings are listed in Table 1.6,7
Originally described by Laennac in 1819,1         There is usually a history of a chronic
bronchiectasis is a chronic, debilitating         productive cough and recurrent chest               Assessment of severity
condition characterised by persistent             infections. There may be symptoms
cough, excessive sputum production and            related to airways obstruction (wheeze             Clinical, radiological and microbiological
recurrent chest infections. The precise           and breathlessness), mucus plugging                features guide clinicians to the severity of
prevalence is unknown, but figures quoted         (chest pain) and also systemic symp-               bronchiectasis. These investigations not
vary from about 4 × 105 aged 18–34 years          toms.4 The diagnosis of bronchiectasis is          only provide clinicians with a quantitative
to 272 × 105 aged 75 years and over.2,3           confirmed radiologically with computed             assessment of disease severity but may
                                                                                                     also help in the management of both
                                                                                                     stable disease and exacerbations.
Key Points                                                                                           •   Sputum colour and volume. Colour is
                                                                                                         graded as mucoid, mucopurulent or
Bronchiectasis should be considered in patients with a chronic, productive cough                         purulent (Fig 2) and volume is
   and a history of recurrent chest infections
                                                                                                         measured over a 24-hour collection
The gold standard for diagnosis is computed tomography of the chest                                      period. Patients with severe
                                                                                                         bronchiectasis usually have purulent
The aetiology is unknown in up to 50% of cases and post-infective in up to 42%                           sputum and volumes that may
The mainstays of treatment are regular chest physiotherapy, annual influenza                             exceed 25 ml/day, even when stable.
  vaccination and prompt administration of antibiotics for exacerbations
                                                                                                     •   Exacerbation frequency. In severe
                                                                                                         disease there are often multiple
Long-term antibiotics should be considered for patients with recurrent chest
  infections impacting on their health-related quality of life                                           exacerbations (usually ≥3 a year)
                                                                                                         and inpatient management may be
KEY WORDS: bronchiectasis, exacerbations, investigations, management                                     necessary.

(a)                                         (b)                                               (c)

Fig 1. (a) Tubular dilation of airways (see arrow); (b) varicose dilatation of airways (arrow shows irregular, dilated airway);
(c) cystic dilatation of airways with thickened bronchi and mucus plugging (see arrow).

164                                                                                               Clinical Medicine Vol 9 No 2 April 2009
                                                                                             © Royal College of Physicians, 2009. All rights reserved.
                                                                                                       CME Respiratory medicine

•        Lung function. There can be                          usually varicose or cystic with                        with severe bronchiectasis are
         advanced airflow obstruction in            
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