Bronchiectasis

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Bronchiectasis
Classification and external resources
Bronchiectasis NHLBI.jpg
Figure A shows a cross-section of the lungs with normal airways and widened airways. Figure B shows a cross-section of a normal airway. Figure C shows a cross-section of an airway with bronchiectasis.
ICD-10J47, Q33.4
ICD-9494, 748.61
DiseasesDB1684
MedlinePlus000144
eMedicinemed/246
MeSHD001987
 
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Bronchiectasis
Classification and external resources
Bronchiectasis NHLBI.jpg
Figure A shows a cross-section of the lungs with normal airways and widened airways. Figure B shows a cross-section of a normal airway. Figure C shows a cross-section of an airway with bronchiectasis.
ICD-10J47, Q33.4
ICD-9494, 748.61
DiseasesDB1684
MedlinePlus000144
eMedicinemed/246
MeSHD001987

Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial tree caused by destruction of the muscle and elastic tissue. It is classified as an obstructive lung disease, along with emphysema, bronchitis, asthma, and cystic fibrosis.

Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airway obstruction and impaired clearance of secretions. Bronchiectasis may result from a variety of infective and acquired causes, including severe and recurrent pneumonia, tuberculosis, and cystic fibrosis.[1]

Signs and symptoms[edit]

Some people with bronchiectasis may produce frequent green/yellow sputum (up to 240ml (8 oz) daily). Bronchiectasis may also present with hemoptysis in the absence of sputum, called "dry bronchiectasis". Sputum production may also occur without coloration. People with bronchiectasis may have bad breath indicative of active infection. Frequent bronchial infections and breathlessness are two possible indicators of bronchiectasis.[1]

Crepitations and expiratory rhonchi may be heard on auscultation, although clubbing is rare.[2]

Causes[edit]

Bronchiectasis has both congenital and acquired causes, with the latter more frequent.[3]

Acquired causes[edit]

Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. The yellowish discoloration of lung parenchyma reflects obstructive pneumonia.

Tuberculosis, pneumonia, inhaled foreign bodies, allergic bronchopulmonary aspergillosis and bronchiol tumours are the major acquired causes of Bronchiectasis.[1][4] Infective causes associated with Bronchiectasis include infections caused by the Staphylococcus, Klebsiella, or Bordetella pertussis, the causative agent of whooping cough.[3]

Aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies all appear to be linked to the development of Bronchiectasis,[5][source needs translation]

Various immunological and lifestyle factors have also been linked to the development of bronchiectasis:

No cause is identified in up to 50% of non-cystic-fibrosis related bronchiecstasis.[2]

Congenital causes[edit]

Bronchiectasis may result from congenital infections that affect cilia motility or ion transport.[1] Kartagener syndrome is one such disorder of cilia motility linked to the development of bronchiectasis,.[9] A common cause is cystic fibrosis, which affects chloride ion transport, in which a small number of patients develop severe localized bronchiectasis.[10] Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic infections of the sinuses and bronchiole tree.[11]

Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung.[12] Several other congenital disorders can also lead to bronchiectasis, including Williams-Campbell syndrome[13] and Marfan syndrome.[14]

Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons.[15]

Pathophysiology[edit]

Bronchiectasis, gross pathology.

Bronchiectasis is a result of chronic inflammation compounded by an inability to clear mucoid secretions. This can be a result of genetic conditions resulting in a failure to clear sputum (Primary ciliary dyskinesia), or resulting in more viscous sputum (cystic fibrosis), or the result of chronic or severe infections. Inflammation results in progressive destruction of the normal lung architecture, in particular the elastic fibres of bronchi.[1]

Tuberculosis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.[16]

Diagnosis[edit]

Bronchiectasis may be diagnosed clinically or on review of imaging.[1][17] The British Thoracic Society recommends all non cystic-fibrosis-related bronchiectasis be confirmed by CT.[2] CT may reveal tree-in-bud abnormalities, dilated bronchi, and cysts with defined borders.[1]

Other investigations typically performed at diagnosis include blood tests, sputum cultures, and sometimes tests for specific genetic disorders.[1]

Prevention[edit]

In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent the development of infections (such as bronchitis) and further complications.[18]

Treatments to slow down the progression of this chronic disease include keeping bronchial airways clear and secretions weakened through various forms of pneumotherapy. Aggressively treating bronchial infections with antibiotics to prevent the destructive cycle of infection, damage to bronchial tubes, and more infection is also standard treatment. Regular vaccination against pneumonia, influenza and pertussis are generally advised. A healthy body mass index and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.[19]

Treatment[edit]

Treatment of bronchiectasis includes controlling infections and bronchial secretions, relieving airway obstructions, removal of affected portions of lung by surgical removal or artery embolization and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections,[20] as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Postural drainage techniques, aided by physiotherapists, are an important mainstay of treatment.[1][2]

Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.[21]

Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time, and help prevent progression of bronchiectasis. This is not recommended for routine use in children.[2] One commonly used therapy is beclometasone dipropionate, which is also used in asthma treatment.[22] Use of inhalers such as albuterol (salbutamol), fluticasone (Flovent/Flixotide) and ipratropium (Atrovent) may help reduce likelihood of infection by clearing the airways and decreasing inflammation.[23]

Although not approved for use in any country, Mannitol dry inhalation powder, under the name Bronchitol, has been granted orphan drug status by the FDA for use in patients with bronchiectasis and with cystic fibrosis.[24]

Additionally, the use of ACBT (Active Cycle Breathing Techniques) can be useful in the clearance of sputum. These techniques encourage relaxed, diaphragmatic breathing, greater expansion (via collateral inflation) of otherwise consolidated areas of the lungs, and help in mucociliary clearance (MCC). A useful adjunct to these cycles are manual techniques, wherein the healthcare professional uses percussion, vibrations, and shaking, to dislodge sputum from the chest walls, enabling the patient to expectorate more easily.[citation needed]

Epidemiology[edit]

History[edit]

Rene Laënnec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819.[25] The disease was researched in greater detail by Sir William Osler in the late 1800s; it is suspected that Osler actually died of complications from undiagnosed bronchiectasis.[26]

See also[edit]

Additional images[edit]

References[edit]

  1. ^ a b c d e f g h i Davidson's principles and practice of medicine. (21st ed. ed.). Edinburgh: Churchill Livingstone/Elsevier. 2010. p. 676. ISBN 978-0-7020-3085-7. 
  2. ^ a b c d e Hill, Adam T; Pasteur, Mark; Cornford, Charles; Welham, Sally; Bilton, Diana (1 January 2011). "Primary care summary of the British Thoracic Society Guideline on the management of non-cystic fibrosis bronchiectasis". Primary Care Respiratory Journal 20 (2): 135. doi:10.4104/pcrj.2011.00007. 
  3. ^ a b Hassan, Isaac (8 December 2006). "Bronchiectasis". eMedicine Specialties Encyclopedia. Gibraltar: WebMD. Retrieved 2007-06-22. 
  4. ^ Allergic Bronchopulmonary Aspergillosis
  5. ^ Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N (2006). "Bronchiectasis and clearence [sic] physiotherapy: emphasis in postural drainage and percussion". Braz. J. Cardiovasc. Surg. (in Portuguese) 21 (2). doi:10.1590/S1678-97412006000200015. 
  6. ^ Sheikh S, Madiraju K, Steiner P, Rao M (1997). "Bronchiectasis in pediatric AIDS". Chest 112 (5): 1202–7. doi:10.1378/chest.112.5.1202. PMID 9367458. 
  7. ^ Ferguson HR, Convery RP (2002). "An unusual complication of ulcerative colitis". Postgrad. Med. J. 78 (922): 503. doi:10.1136/pmj.78.922.503. PMC 1742448. PMID 12185236. 
  8. ^ Kaushik, VV, Hutchinson D, Desmond J, Lynch MP, and Dawson JK (2004). "Association between bronchiectasis and smoking in patients with rheumatoid arthritis". Annals of the Rheumatic Diseases 63 (8): 1001–2. doi:10.1136/ard.2003.015123. PMC 1755104. PMID 15249329. 
  9. ^ Morillas HN, Zariwala M, Knowles MR (2007). "Genetic Causes of Bronchiectasis: Primary Ciliary Dyskinesia". Respiration 72 (3): 252–63. doi:10.1159/000101783. PMID 17534128. 
  10. ^ Dalrymple-Hay MJ, Lucas J, Connett G, Lea RE (1999). "Lung resection for the treatment of severe localized bronchiectasis in cystic fibrosis patients". Acta Chir Hung. 38 (1): 23–5. PMID 10439089. 
  11. ^ Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR (1984). "Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections". NEJM 310 (1): 3–9. doi:10.1056/NEJM198401053100102. PMID 6689737. 
  12. ^ Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP (2007). "Genetic causes of bronchiectasis: primary immune deficiencies and the lung". Respiration + 74 (3): 264–75. doi:10.1159/000101784. PMID 17534129. 
  13. ^ WILLIAMS H, CAMPBELL P (April 1960). "Generalized Bronchiectasis associated with Deficiency of Cartilage in the Bronchial Tree". Arch. Dis. Child. 35 (180): 182–91. doi:10.1136/adc.35.180.182. PMC 2012546. PMID 13844857. 
  14. ^ "Medical Problems and Treatments | The Marfan Trust". The Marfan Trust. Retrieved 2010-12-08. 
  15. ^ Shin MS, Ho KJ (1993). "Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?". Chest 104 (5): 1384–86. doi:10.1378/chest.104.5.1384. PMID 8222792. 
  16. ^ Catanzano, Tara (5 September 2005). "Primary Tuberculosis". eMedicine Specialties Encyclopedia. Connecticut: WebMD. Retrieved 2007-06-22. 
  17. ^ Miller, JC (2006). "Pulmonary Mycobacterium Avium-Intracellular Infections in Women". Radiology Rounds 4 (2). 
  18. ^ Crofton J (1966). "Diagnosis and Treatment of Bronchiectasis: I. Diagnosis". Br Med J 1 (5489): 721–3 contd. doi:10.1136/bmj.1.5489.721. PMC 1844268. PMID 5909486. 
  19. ^ Onen ZP, Eris Gulbay B, Sen E, Akkoca Yildiz O, Saryal S, Acican T, Karabiyikoglu G (2007). "Analysis of the factors related to mortality in patients with bronchiectasis". Respir Med. 101 (7): 1390–97. doi:10.1016/j.rmed.2007.02.002. PMID 17374480. 
  20. ^ Evans DJ, Bara AI,Greenstone M (2007). "Prolonged antibiotics for purulent bronchiectasis in children and adults". In Evans, David J. Cochrane Database Syst Rev (2): CD001392. doi:10.1002/14651858.CD001392.pub2. PMID 17443506. 
  21. ^ Otgün I, Karnak I, Tanyel FC, Senocak ME, Büyükpamukçu N (2004). "Surgical treatment of bronchiectasis in children". J. Pediatr. Surg. 39 (10): 1532–6. doi:10.1016/j.jpedsurg.2004.06.009. PMID 15486899. 
  22. ^ Elborn JS, Johnston B, Allen F, Clarke J, McGarry J, Varghese G. (1992). "Inhaled steroids in patients with bronchiectasis". Respir Med 86 (2): 121–4. doi:10.1016/S0954-6111(06)80227-1. PMID 1615177. 
  23. ^ Reports, Consumer (15 March 2007). "Ipratropium and Albuterol Inhalation - Drug Review". Consumer Reports of U.S. Retrieved 2007-06-22. 
  24. ^ Waknine, Yael (27 July 2005). "Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040". Medscape today for WebMD. Retrieved 2007-06-22. 
  25. ^ Roguin, A (2006). "Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope". Clin Med Res 4 (3): 230–35. doi:10.3121/cmr.4.3.230. PMC 1570491. PMID 17048358. 
  26. ^ Wrong O (2003). "Osler and my father". J R Soc Med 96 (6): 462–64. doi:10.1258/jrsm.96.9.462. PMC 539606. PMID 12949207. 

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