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bronchiectasis

bronchiectasis

introduction

  • defined by localized, irreversible dilation of part of the bronchial tree caused by destruction of the muscle and elastic tissue
  • bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions.
  • causes productive chronic cough usually with yellow-green sputum which is often copious and causes halitosis
  • diagnosis can be confirmed by high resolution CT scan

epidemiology

  • poor data on prevalance but it is more common in lower socioeconomic classes and indigenous communities
  • in USA, 4 per 100,000 in ages 18-34yrs rising to nearly 300 per 100,000 over age 75yrs, but UK studies found it may be almost double this for women over age 60yrs

aetiology

  • cystic fibrosis
  • bacterial lung infections
  • HIV / AIDS - the leading cause in children worldwide 1)
    • can occur after a primary TB episode but usually has other features such as calcified hilar lymph nodes, calcified parenchymal granulomas, and upper zone volume loss, and the bronchiectasis tends to be assymetric upper lobar
    • tree-in-bud nodules and cavities are clues to active infection
  • Lady Windermere Syndrome - non-TB mycobacteria infecting R middle or L. lingula lobes, mainly in middle aged or elderly thin women who avoid expectorating for social reasons and fail to clear the sputum.
  • rheumatoid arthritis patients who smoke
  • pulmonary aspiration
  • allergic bronchopulmonary aspergillosis
    • CT findings include include cystic or varicoid usually central bronchiectasis, a tree-in-bud pattern of nodules, bronchial wall thickening, and airtrapping with a central or proximal upper lobe predominance
    • usually central bronchiectasis with prominant LNs
    • pulm. sarcoidosis mainly affects upper and mid zones of lungs
  • immotile cilia syndrome (ICS):
    • autosomal recessive genetic condition with extensive genetic heterogeneity characterized by abnormal ciliary motion and impaired mucociliary clearance (see also ciliopathies)
    • this leads to recurrent or persistent respiratory infections, sinusitis, otitis media, and male infertility.
    • in 50% of the patients, ICS is associated with situs inversus and such patients are classified as having Kartagener's syndrome:
      • autosomal recessive genetic condition with triad of situs inversus, chronic sinusitis, and bronchiectasis
      • symptoms result from defective cilia motility
  • Young's syndrome
    • rare condition of epididymal obstructive azoospermia, infertility and chronic respiratory disorders in middle aged men
    • accounts for 3% of bronchiectasis in a UK study in 2000 (presumably most were born before 1955?) and perhaps half of all cases of azospermia in the late 1960's
    • some believe there may be an association with mercury exposure in childhood (eg. mercurous chloride (or “calomel”) which was removed from teething powders and worm medication in 1955)2)
    • seems to be a separate entity to cystic fibrosis3)
    • could it just be part of immotile cilia syndrome, although no defect in cilia beating has been shown?
  • alpha 1-antitrypsin deficiency
  • primary immunodeficiencies

Other references

bronchiectasis.txt · Last modified: 2018/11/17 12:58 (external edit)