Bronchiectasis: Difference between revisions
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor-in-Chief: Piali Mukherjee
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Overview
Bronchiectasis is a disease that causes localized, irreversible dilatation of part of the bronchial tree. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions. Bronchiectasis is associated with a wide range of disorders, but it usually results from necrotizing bacterial infections, such as infections caused by the Staphylococcus or Klebsiella species or Bordetella pertussis.[1]
Rene Theophile Hyacinthe Laënnec, the man who invented the stethoscope, used his creation to first discover bronchiectasis in 1819.[2]. The disease was researched in greater detail by Sir William Osler in the late 1800s; in fact, it is suspected that Osler actually died of complications from undiagnosed bronchiectasis[3].
Pathogenesis
Dilation of the bronchial walls results in airflow obstruction and impaired clearance of secretions because the dilated areas interrupt normal air pressure of the bronchial tubes, causing sputum to pool inside the dilated areas instead of being pushed upward[4]. The pooled sputum provides an environment conducive to the growth of infectious pathogens, and these areas of the lungs are thus very vulnerable to infection. The more infections that the lungs experience, the more damaged the lung tissue and alveoli become. When this happens, the bronchial tubes become more inelastic and dilated, which creates a perpetual, destructive cycle within this disease.
There are three types of brochiectasis, varying by level of severity. Fusiform (cylindrical) bronchiectasis (the most common type) refers to mildly inflamed bronchi that fail to taper distally. In varicose bronchiectasis, the bronchial walls appear beaded, because areas of dilation are mixed with areas of constriction. Saccular (cystic) bronchiectasis is characterized by severe, irreversible ballooning of the bronchi peripherally, with or without air-fluid levels.[5] Chronic productive cough is prominent, occurring in up to 90% of patients with bronchiectasis. Sputum is produced on a daily basis in 76% of patients.[6]
Causes
There are both congenital and acquired causes of bronchiectasis. Kartagener syndrome, which affects the mobility of cilia in the lungs[7], aids in the development of the disease. Another common genetic cause is Cystic Fibrosis, in which a small number of patients develop severe localized bronchiectasis[8]. Young's syndrome, which is clinically similar to Cystic Fibrosis, is thought to significantly contribute to the develop of Bronchiectasis. This is due to the occurrence of chronic, sinopulmonary infections.[9] Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons. [10] 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.[11]
Acquired bronchiectasis occurs more frequently, with one of the biggest causes being tuberculosis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.[12] A especially common cause of the disease in children is Acquired Immunodeficiency Syndrome, stemming from the human immunodeficiency virus. This disease predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.[13]. Bronchiectasis can sometimes be an unusual complication of Inflammatory bowel disease, especially Ulcerative Colitis. It can occur in Crohn's Disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungus spores.[14] Recent evidence has shown an increased risk of Bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold increased prevalence of the disease in this cohort[15]. Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.
Other acquired causes of bronchiectasis involving environmental exposures include respiratory infections, obstructions, inhalation and aspiration of ammonia and other toxic gases, Pulmonary aspiration, alcoholism, heroin (drug use), and various allergies.[16]
Diagnosis
The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of 80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan confirmation if clinical history clearly demonstrates frequent, respiratory infections, as well confirmation of an underlying problem via blood work and sputum culture samples.[17]
Treatment
Treatment of bronchiectasis is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications. This includes prolonged usage of antibiotics to prevent detrimental infections[18], as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.[19]
Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time will prevent progression of bronchiectasis. One commonly used therapy is Beclometasone dipropionate, also used in asthma treatment.[20] 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.[21]
Mannitol dry inhalation powder, under the name Bronchitol, has been approved by the FDA for use in Cystic Fibrosis patients with Bronchiectasis. The original orphan drug indication approved in February 2005 allowed its use for the treatment of bronchiectasis. The original approval was based on the results of phase 2 clinical studies showing the product to be safe, well-tolerated, and effective for stimulating mucus hydration/clearance, thereby improving quality of life in patients with chronic obstructive lung diseases like Bronchiectasis. Long-term studies are currently underway to ensure the safety and effectiveness of the treatment, and it is not yet available on the market for use.[22]
Prevention
In order to prevent future development of bronchiectasis, an x-ray of the chest should be taken after any severe attack of measles, whooping cough or other acute respiratory infection in 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.[23]
A healthy Body Mass Index, vaccination (especially against pneumonia and influenza) 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.[24]
References
- ↑ Hassan, Isaac (December 8, 2006). "Bronchiectasis". eMedicine Specialties Encyclopedia. Gibraltar: WebMD.
- ↑ Roguin, A (2006). "Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope". Clin Med Res. 4 (3): 230–35.
- ↑ Wrong O (2003). "Osler and my father". J R Soc Med. 96 (6): 462–64.
- ↑ Morrissey BM (2007). "Pathogenesis of bronchiectasis". Clin Chest Med. 28 (2): 289–96. PMID 17467548.
- ↑ Mysliwiec, V, Pina, JS (1999). "Bronchiectasis: the 'other' obstructive lung disease". POSTGRADUATE MEDICINE. 106 (1): 252–63.
- ↑ Emmons, Ethan (January 31, 2007). "Bronchiectasis". eMedicine Specialties Encyclopedia. San Antonio, TX: WebMD.
- ↑ Morillas HN, Zariwala M, Knowles MR (2007). "Genetic Causes of Bronchiectasis: Primary Ciliary Dyskinesia". Respiration. 72 (3): 252–63. PMID 17534128.
- ↑ 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.
- ↑ Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR (1984). "Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections". NEJM. 310 (1): 3–9.
- ↑ Shin MS, Ho KJ (1993). "Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?". Chest. 104: 1384–86.
- ↑ 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. PMID 17534129.
- ↑ Catanzano, Tara (September 5, 2005). "Primary Tuberculosis". eMedicine Specialties Encyclopedia. Connecticut: WebMD.
- ↑ Sheikh S, Madiraju K, Steiner P, Rao M (1997). "Bronchiectasis in pediatric AIDS". Chest. 112 (5): 1202–7. PMID 9367458.
- ↑ Ferguson HR, Convery RP (2002). "An unusual complication of ulcerative colitis". Postgrad. Med. J. 78: 503.
- ↑ 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: 1001–2.
- ↑ Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N (2006). "Bronchiectasis and clearence physiotherapy: emphasis in postural drainage and percussion". Braz. j. cardiovasc. surg. (in Portuguese). 21 (2).
- ↑ Miller, JC (2006). "Pulmonary Mycobacterium Avium-Intracellular Infections in Women". Radiology Rounds. 4 (2).
- ↑ Evans DJ, Bara AI,Greenstone M (2007). "Prolonged antibiotics for purulent bronchiectasis in children and adults". The Cochrane Database of Systematic Reviews (2). doi:10.1002/14651858.CD001392.pub2.
- ↑ Ötgün B, Karnak B, Tanyel K, Enocak M, Büyükpamukçu N (2003). "Surgical treatment of bronchiectasis in children". Journal of Pediatric Surgery. 39 (10): 1532–36.
- ↑ 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. PMID 1615177.
- ↑ Reports, Consumer. "Ipratropium and Albuterol Inhalation - Drug Review". Consumer Reports of U.S.
- ↑ Waknine, Yael. "Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040". Medscape today for WebMD.
- ↑ Crofton J (1966). "Diagnosis and Treatment of Bronchiectasis: II. Treatment and Prevention". Br Med J. 1 (5490): 783–785.
- ↑ 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. PMID 17374480.
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