Bronchiectasis 331633 226043747 2008-07-16T16:02:13Z Thijs!bot 1392310 robot Adding: [[eo:Bronkektazio]] {{DiseaseDisorder infobox | Name = Bronchiectasis | Image = Illu_bronchi_lungs.jpg| Caption = | ICD9 = {{ICD9|494}}, {{ICD9|748.61}} | ICD10 = {{ICD10|J|47||j|40}}, {{ICD10|Q|33|4|q|30}}| OMIM = | OMIM_mult = | MedlinePlus = 000144 | eMedicineSubj = med | eMedicineTopic = 246 | DiseasesDB = 1684 | MeshID = D001987 | }} '''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 [[Airway obstruction|airflow obstruction]] and impaired clearance of secretions. Bronchiectasis is associated with a wide range of disorders, but it usually results from [[Necrotizing fascitis|necrotizing]] bacterial infections, such as infections caused by the ''[[Staphylococcus]]'' or ''[[Klebsiella]]'' species or ''[[Bordetella pertussis]]''.<ref>{{cite encyclopedia| last = Hassan | first = Isaac | authorlink = | title = Bronchiectasis | encyclopedia = eMedicine Specialties Encyclopedia | volume = | pages = | publisher = WebMD | location = Gibraltar | date = 2006-12-08 | url = http://www.emedicine.com/radio/topic116.htm | accessdate = 2007-06-22}}</ref> [[René Laënnec|Rene Theophile Hyacinthe Laënnec]], the man who invented the [[stethoscope]], used his creation to first discover bronchiectasis in [[1819]].<ref>{{cite journal |author=Roguin, A |title=Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope |language=English |journal=Clin Med Res|volume=4 |issue=3 |pages=230–35 |year=2006 |pmid= |doi=}}</ref>. 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<ref>{{cite journal |author=Wrong O |title=Osler and my father|language=English |journal=J R Soc Med |volume=96 |issue=6 |pages=462–64 |year=2003 |pmid= 12949207|doi=10.1258/jrsm.96.9.462}}</ref>. == Pathogenesis == Dilation of the bronchial walls results in airflow obstruction and impaired clearance of secretions because the dilated areas disrupt normal air pressure in the bronchial tubes, causing [[sputum]] to pool inside the dilated areas instead of being pushed upward<ref>{{cite journal |author=Morrissey BM |title=Pathogenesis of bronchiectasis |language=English |journal=Clin Chest Med|volume=28 |issue=2 |pages=289–96 |year=2007 |pmid=17467548|doi=10.1016/j.ccm.2007.02.014}}</ref>. The pooled sputum provides an environment conducive to the growth of infectious [[pathogen]]s, 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, creating a self-perpetuating cycle of further damage to the lungs. 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 [[Anatomical terms of location#Proximal and distal|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 and irreversible ballooning of the bronchi peripherally, with or without air-fluid levels.<ref>{{cite journal |author=Mysliwiec, V, Pina, JS |title=Bronchiectasis: the 'other' obstructive lung disease |language=English |journal=Postgraduate Medicine |volume=106 |issue=1 |pages=252–63 |year=1999 |pmid= |doi=}}</ref> 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.<ref>{{cite encyclopedia| last = Emmons | first = Ethan | authorlink = | title = Bronchiectasis | encyclopedia = eMedicine Specialties Encyclopedia | volume = | pages = | publisher = WebMD | location = San Antonio, TX | date = 2007-01-31 | url = http://www.emedicine.com/med/topic246.htm | accessdate = 2007-06-22}}</ref> Generally, persons suffering from bronchiectasis tend to be infected by ''[[Haemophilus influenzae]]'' early on in the disease course. Secondary infection is usually due to ''[[Staphylococcus aureus]]''; followed by ''[[Moraxella catarrhalis]]'' and finally ''[[Pseudomonas aeruginosa]]''.<ref name="oherm">{{cite book | last = Parker | first = Robert | coauthors = Catherine Thomas, Lesley Bennett, ''et al'' | title = Emergencies in Respiratory Medicine | publisher = Oxford | date = 2007 | pages = 206-207 | isbn = 978-0-19-920244-7 }}</ref> ==Causes== There are both [[congenital]] and [[acquired]] causes of bronchiectasis. [[Primary ciliary dyskinesia|Kartagener syndrome]], which affects the mobility of [[cilia]] in the lungs<ref>{{cite journal |author=Morillas HN, Zariwala M, Knowles MR|title=Genetic Causes of Bronchiectasis: Primary Ciliary Dyskinesia |language=English |journal=Respiration |volume=72 |issue=3 |pages=252–63 |year=2007 |pmid=17534128 |doi=}}</ref>, 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<ref>{{cite journal |author=Dalrymple-Hay MJ, Lucas J, Connett G, Lea RE|title=Lung resection for the treatment of severe localized bronchiectasis in cystic fibrosis patients|language=English |journal=Acta Chir Hung. |volume=38 |issue=1 |pages=23–5 |year=1999 |pmid=10439089 |doi=}}</ref>. [[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]], [[sinopulmonary]] infections.<ref>{{cite journal |author=Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR |title=Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections |language=English |journal=NEJM |volume=310 |issue=1 |pages=3–9 |year=1984 |pmid= |doi=}}</ref> Patients with [[alpha 1-antitrypsin]] deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons. <ref>{{cite journal |author=Shin MS, Ho KJ |title=Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence? |language=English |journal=Chest |volume=104 |issue= |pages=1384–86 |year=1993|doi=10.1378/chest.104.5.1384}}</ref> 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.<ref>{{cite journal |author=Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP |title=Genetic causes of bronchiectasis: primary immune deficiencies and the lung |language=English |journal=Respiration |volume=74 |issue=3 |pages=264–75 |year=2007 |pmid=17534129|doi=10.1159/000101784}}</ref> 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 bronchiectasis|traction]] from fibrosis.<ref>{{cite encyclopedia| last = Catanzano | first = Tara | authorlink = | title = Primary Tuberculosis | encyclopedia = eMedicine Specialties Encyclopedia | volume = | pages = | publisher = WebMD | location = Connecticut | date = 2005-09-05 | url = http://www.emedicine.com/radio/topic411.htm | accessdate = 2007-06-22}}</ref> An especially common cause of the disease in children is [[AIDS|acquired immune deficiency syndrome]], stemming from [[HIV|the human immunodeficiency virus]]. This disease predisposes patients to a variety of pulmonary ailments, such as [[pneumonia]] and other [[opportunistic infection]].<ref>{{cite journal |author=Sheikh S, Madiraju K, Steiner P, Rao M |title=Bronchiectasis in pediatric AIDS|language=English |journal=Chest |volume=112 |issue=5 |pages=1202–7 |year=1997 |pmid=9367458|doi=10.1378/chest.112.5.1202}}</ref>. 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.<ref>{{cite journal |author=Ferguson HR, Convery RP |title=An unusual complication of ulcerative colitis |language=English |journal=Postgrad. Med. J. |volume=78 |issue= |pages=503 |year=2002 |pmid= 12185236|doi=10.1136/pmj.78.922.503}}</ref> 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<ref>{{cite journal |author=Kaushik, VV, Hutchinson D, Desmond J, Lynch MP, and Dawson JK |title=Association between bronchiectasis and smoking in patients with rheumatoid arthritis|language=English |journal=Annals of the Rheumatic Diseases |volume=63|issue= |pages=1001–2 |year=2004 |pmid= 15249329|doi=10.1136/ard.2003.015123}}</ref>. 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, [[Airway obstruction|obstructions]], inhalation and aspiration of [[ammonia]] and other toxic gases, [[pulmonary aspiration]], [[alcoholism]], [[heroin]] (drug use), and various [[allergy|allergies]].<ref>{{cite journal |author=Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N |title=Bronchiectasis and clearence physiotherapy: emphasis in postural drainage and percussion|language=Portuguese |journal=Braz. j. cardiovasc. surg. |volume=21 |issue=2 |pages= |year=2006 |pmid= |doi=}}</ref> ==Diagnosis== The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution [[Computed tomography|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 (tests)|sensitivity]] of 80%, [[Specificity (tests)|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 test|blood work]] and [[sputum]] culture samples.<ref>{{cite journal |author=Miller, JC |title=Pulmonary Mycobacterium Avium-Intracellular Infections in Women |language=English |journal=Radiology Rounds |volume=4 |issue=2 |pages= |year=2006 |pmid= |doi=}}</ref> ==Treatment== Treatment of bronchiectasis is aimed at controlling [[infection]]s and bronchial secretions, relieving airway obstruction, and preventing [[Complication (medicine)|complication]]s. This includes the prolonged usage of [[antibiotic]]s to prevent detrimental infections<ref>{{cite journal |author=Evans DJ, Bara AI,Greenstone M |title=Prolonged antibiotics for purulent bronchiectasis in children and adults |language=English |journal=The Cochrane Database of Systematic Reviews |volume= |issue=2 |pages= |year=2007|doi=10.1002/14651858.CD001392.pub2}}</ref>, 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.<ref>{{cite journal |author=Ötgün B, Karnak B, Tanyel K, Enocak M, Büyükpamukçu N|title=Surgical treatment of bronchiectasis in children |language=English |journal=Journal of Pediatric Surgery |volume=39 |issue=10 |pages=1532–36 |year=2003 |pmid= |doi=}}</ref> 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. One commonly used therapy is [[beclometasone dipropionate]], which also used in [[asthma]] treatment.<ref>{{cite journal |author=Elborn JS, Johnston B, Allen F, Clarke J, McGarry J, Varghese G. |title=Inhaled steroids in patients with bronchiectasis |language=English |journal=Respir Med |volume=86 |issue=2 |pages=121–4 |year=1992 |pmid=1615177|doi=10.1016/S0954-6111(06)80227-1}}</ref> Use of [[Metered-dose inhaler|inhalers]] such as [[Salbutamol|albuterol (salbutamol)]], [[Fluticasone|fluticasone (Flovent/Flixotide)]] and [[Ipratropium|ipratropium (Atrovent)]] may help reduce likelihood of infection by clearing the airways and decreasing inflammation.<ref>{{cite web | last = Reports | first = Consumer | authorlink = | coauthors = | title = Ipratropium and Albuterol Inhalation - Drug Review | work = | publisher = Consumer Reports of U.S.| date = [[2007-03-15]] | url = http://www.consumerreports.org/mg/drug-reports/ipratropium-and-albuterol-inhalation.htm | format = | doi = | accessdate = 2007-06-22}}</ref> [[Mannitol]] dry inhalation powder, under the name Bronchitol, has been approved by the [[Food and Drug Administration|FDA]] for use in cystic fibrosis patients with or at risk for 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 II [[clinical trial|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 underway as of 2007 to ensure the safety and effectiveness of the treatment.<ref>{{cite web | last = Waknine | first = Yael | authorlink = | coauthors = | title = Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040 | work = | publisher = Medscape today for WebMD | date = [[2005-07-27]] | url = http://www.medscape.com/viewarticle/509116 | format = | doi = | accessdate = 2007-06-22}}</ref> Advair Diskus is also a commonly used inhaled corticosteroid which has in many cases been effective in clearing the airways, reducing sputum and reducing inflammation. ==Prevention== In order to prevent future development of bronchiectasis, an [[Chest X-ray|x-ray]] of the chest should be taken after any severe attack of [[measles]], [[Pertussis|whooping cough]] or other acute respiratory infection in childhood. While [[Tobacco smoking|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.<ref>{{cite journal |author=Crofton J|title=Diagnosis and Treatment of Bronchiectasis: II. Treatment and Prevention |language=English |journal= Br Med J |volume=1 |issue=5490 |pages=783–785 |year=1966 |pmid= |doi=}}</ref> 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.<ref>{{cite journal |author=Onen ZP, Eris Gulbay B, Sen E, Akkoca Yildiz O, Saryal S, Acican T, Karabiyikoglu G|title=Analysis of the factors related to mortality in patients with bronchiectasis |language=English |journal= Respir Med. |volume=101 |issue=7 |pages=1390–97 |year=2007 |pmid=17374480|doi=10.1016/j.rmed.2007.02.002}}</ref> ==See also== *[[Traction bronchiectasis]] == References == {{reflist|2}} == External links == {{Wikisource1911Enc|Bronchiectasis}} * {{MerckHome|04|047|a}} * {{MerckManual|6|70|a}} * {{GPnotebook|-214630398}} * [http://myweb.lsbu.ac.uk/~dirt/museum/p6-26.html#Analysis Imaging] {{Respiratory pathology}} {{Congenital malformations and deformations of respiratory system}} </sup> [[Category:Diseases]] [[Category:Pulmonology]] [[de:Bronchiektasie]] [[es:Bronquiectasia]] [[eo:Bronkektazio]] [[fr:Bronchectasie]] [[ko:기관지확장증]] [[it:Bronchiectasia]] [[nl:Bronchiëctasieën]] [[ja:気管支拡張症]] [[nn:Bronkiektasi]] [[pl:Rozstrzenie oskrzeli]] [[sv:Bronkiektasier]] [[tr:Bronşektazi]]