[Downloaded free from http://www.thoracicmedicine.org on Friday, June 10, 2016, IP: 27.34.246.

Review Article

Pathogenesis, etiology and treatment of
Nehad Al-Shirawi1, Hamdan H. Al-Jahdali2, Abdullah Al Shimemeri2

Critical Care Bronchiectasis is a chronic lung disease, defined pathologically as irreversible dilatation of the bronchi. The
Department, clinical course of the disease is chronic and progressive and in most cases, causes lung damage over many
Department King years. There is usually an initial event, which causes impairment of mucociliary clearance of the bronchial tree.
Abdul-Aziz Medical The respiratory tract becomes colonized by bacteria that inhibit the ciliary function and promote further lung
damage. The hallmark of bronchiectasis, is a chronic cough with mucopurulent or purulent sputum, lasting for
City, King Fahad months to years and may progress to chronic respiratory failure. Diagnosis of bronchiectasis is suspected on
National Guard the basis of clinical manifestations. In order to confirm the diagnosis and underlying causes, appropriate
Hospital, (KAMC- investigations must be performed. In this comprehensive review, we discuss the etiology, pathogenesis, clinical
KFNGH), Saudi Arabia presentation, appropriate investigations and management of bronchiectasis.
Pulmonary Section, Key words:
Department of Bronchiectasis, chronic lung disease.
Medicine, King Abdul-
Aziz Medical City and
College of Medicine,
ronchiectasis is a chronic lung disease that is called varicose bronchiectasis and is
King Saud Bin Abdul-
Aziz University for
Health Science
B is characterized by permanent dilatation of
the bronchi and fibrosis of the lung.[1-3] The true
characterized by irregular and beaded outline
of bronchi, with alternating areas of constriction
prevalence of bronchiectasis is difficult to and dilatation. The third type is called cystic or
determine, as a result of several factors.[1] One saccular bronchiectasis and is the most severe
of the factors, is the under-investigation of the form of the disease. The bronchi dilate, forming
disease in patients, with a known cause for large cysts, which are usually filled with air and
chronic sputum production e.g., Smokers.[1] fluid. The fourth type of bronchiectasis is called
Previously, bronchiectasis was a very common follicular and is characterized by extensive
chronic pulmonary problem; however, lymphoid nodules within the bronchial walls.
currently, the prevalence of the disease is It usually occurs following childhood infections.
decreasing.[4-8]The decline in the prevalence However, the clinical usefulness of designating
could be the result of early treatment of mild bronchiectasis to one of these patterns is
cases, effective anti-tuberculous therapy and questionable and no study to date, has shown a
immunization against pertussis and measles.[4­ clinical, epidemiologic, or pathophysiologic
The prevalence of bronchiectasis worldwide, difference between these patterns.[12] [Table 1]
is unknown. In the United States, bronchiectasis summarizes the types of bronchiectasis.
is still prevalent in certain populations, with Bronchiectasis can present as either local
high rates of childhood respiratory tract disease, or a diffuse process involving both
infections and poor access to healthcare lungs.[4] Focal bronchiectasis may be the result
facilities.[9] The prevalence of bronchiectasis in of blockage of the bronchial lumen by a foreign
Correspondence to: Saudi Arabia, is not well studied. In a study by body, tumour, or as a result of extrinsic
Dr. Hamdan Al-Jahdali, Al-Mobeireek et al, bronchiectasis was found to compression of the bronchi. The middle lobe
Pulmonary Section,
represent only 5% of the causes of chronic syndrome is an example of focal bronchiectasis
Department of Medicine,
King Abdul-Aziz Medical persistent cough in the adult population, caused by extrinsic compression of the bronchi,
City, Mail Code 1443, referred to a pulmonary clinic.[10] by enlarged lymph node secondary to
King Fahad National mycobacterial or fungal infection.[13] Diffuse
Guard Hospital, P.O. Box Pathological types of Bronchiectasis bronchiectasis is usually caused either by
22490, Riyadh - 11426, congenital disease, or in association with
Kingdom of Saudi Arabia. Pathologically, bronchiectasis can be divided systemic diseases.[4,12]
into four types.[4,11] The first type, cylindrical
bronchiectasis, is characterized by uniform Clinical Presentation
Submission: 15-1-2006 dilatation of bronchi, that extends into the lung
Accepted: 25-3-2006 periphery, without tapering. The second type Clinically, most patients present with a long-

Annals of Thoracic Medicine - Vol 1, Issue 1, June 2006 41

thoracicmedicine. are associated with bronchiectasis. Allergic bronchpulmonary aspergillosis of bronchiectasis. Others have chronic • Others production of large amounts of mucoid sputum.[1] bronchiectasis and controls. that may be due to peripheral bronchiectasis.[20-21] Whether IgG subclass deficiency leads and the majority will be classified as idiopathic. immune reaction to aspergillus and the action of several mediators. IgM and IgA. that fail to taper o Measles distally o Adenovirus 21 Varicose Beaded bronchial walls. adenovirus 21. as a permanent airway damage.[1. caused by these two infections.140] Al-Shirawi. that turns o Rheumatoid arthritis purulent. the gene frequency of α1.34. that eventually end bronchiectasis will have an obvious cause for their condition in bronchiectasis. etiology and treatment of bronchiectasis Table 1: Pathological types of bronchiectasis. complement deficiency and chronic deficiency was not different between patients with granulomatous disease. Other symptoms include. with or without air fluid o HIV infection levels • Immune dysfunction Follicular Extensive lymphoid nodules within the o Primary and secondary immunoglobulin deficiency bronchial walls o Complement deficiency o Chronic granulomatous disease standing history of either persistent or intermittent. is declining due to idiopathic bronchiectasis. in the absence of emphysema. including direct invasion of the bronchiectasis.antitrypsin deficiency. the cystic fibrosis transmembrane regulator Bronchiectasis. that IgG subclass deficiency may have a role in Infections bronchiectasis development. during infective episodes. is shown to occur in high frequency in children with secondary to mycobarium tuberculosis.4. mycobarium cannot be responsible for bronchiectasis.3] o Toxic chemicals o Herion Causes of Bronchiectasis o Inhaled gastric contents o Foreign body Although many patients seem to have no associated disease o Pulmonary fibrosis that lead to the development of bronchiectasis. may all lead to Cystic fibrosis is well known to cause bronchiectasis. pertussis.[27-30] However.[4] aeruginosa. irreversible ballooning of the o Mycobactrium avium complex (MAC) bronchi peripherally. in a Immunodeficiency syndromes such as immunoglobulin study by Cuvelier et al. put the patient at increased bronchiectasis in patients with a1. o a1.3-4] Sputum could be mucoid. Currently. constitutional o Williams-Campbell syndrome symptoms such as fever. mucopurulent or o Cystic fibrosis viscous.[3-4] Approximately 50% of patients have pleuritic chest o Mounier-Kuhn syndrome • Clearance defects pain. environmental) to contribute to factors may lead to this.[25] However.[26] It is suggested that CFTR mutation acts is associated with airway damage and bronchiectasis.[31] It has been suggested that Deficiency of IgG. [Table 2] lists to bronchiectasis in the presence of near-normal levels of total some of these conditions. is still a controversial issue.antitrypsin deficiency. [1. sputum • Other inherited diseases production. aspergillosis Cystic fibrosis and human immunodeficiency virus (HIV).[4] Hemoptysis does occur and may range from minor. Several with other factors (genetic. Lately.[22-23] Many infections have been implicated to cause bronchiectasis. June 2006 . some reports suggest.4.org on Friday. CFTR mutations alone effective antituberculous treatment. as the heterozygotes avium-intracellulare complex (MAC). Issue 1. Type Description • Infections o Pertussis Cylindrical (fusiform) Uniformly dilated bronchi.12] Less than 40% of patients with risk of recurrent pulmonary infections. is 42 Annals of Thoracic Medicine .[25] airways by the fungus. o Crohn’s disease o Yellow nail syndrome will have their disease progressing to chronic respiratory o Celiac disease failure and will develop cor-pulmonale. (CFTR). as a result of areas o Tuberculosis of dilation. However. 2016. o Kartagener’s syndrome Some individuals with mild disease are completely o Young’s syndrome asymptomatic between exacerbations. loss of appetite and shortness of o Swyer-James syndrome breath. Table 2: Conditions associated with bronchiectasis.antitrypsin deficiency to life threatening. et al .[15-16] α1 – antitrypsin deficiency Bronchiectasis has been reported to occur in α1. IP: 27.[24] In addition.14-19] Immunizations against result of recurrent respiratory tract infections with measles and pertussis have led to marked reduction in the Staphylococcus aureus and mucoid pseudomonas incidence of bronchiectasis. IgG.[4] The clinical spectrum of the disease is broad. mixed with areas of constriction o Aspergillosis Saccular (cystic) Severe. [Downloaded free from http://www. Measles. the incidence of bronchiectasis. tuberculosis. the gene responsible for cystic Tuberculosis was among the most important causes of fibrosis (CF).[1. [1. has been recognized to for this gene mutation were not found to be at increased risk cause bronchiectasis.: Pathogenesis. including Interleukins. or distal o Immotile cilia pneumonitis. June 10.246.antitrypsin Immune dysfunction deficiency. there are many o Absence of bronchial cartilage conditions that have been recognized to cause bronchiectasis.Vol 1.[1] The most severely o Sjogren’s syndrome affected subjects usually have continuous purulent sputum o Ulcerative colitis production and a proportion of those with chronic symptoms.

[41] colonization. as exhaled nitric oxide is reduced in bronchiectatic patients with the inflammatory and autoimmune processes shift from the Pseudomonas aeruginosa infection.[32-33] mucociliary clearance and excess sputum production.org on Friday.140] Al-Shirawi. [1-3] In the Rheumatoid arthritis majority. are in patients with rheumatoid arthritis.3% in persistent. et al .[58] identify. it can induce cytotoxic effects on the bronchial inflammatory bowel disease. bacterial colonization and presence of markers of The association between rheumatoid arthritis and inflammation in the sputum. 2016. that results in arthritis was 5. Idiopathic bronchiectasis represents about half of the cases. [Downloaded free from http://www. Issue 1. develop bronchiectasis after pathogenesis of many respiratory diseases. causing emphysema and adult respiratory distress syndrome. as a result of recurrent from a continuous “vicious circle” of inflammation and tissue pulmonary infections due to retained secretions. It consists of sinusitis. susceptible to microbial colonization. including albumin. etiology and treatment of bronchiectasis caused by emphysema.[1. rather than by α1-antitrysin deficiency the pulmonary defense mechanisms. there is a strong rheumatoid arthritis and bronchiectasis.[12] The majority of cases reported. if of neutrophil products. Although all patients with bronchiectasis have impaired bronchiectasis and situs inversus.[47] with the 24-h sputum volume in these patients. This association is still can explain the worse lung function in patients who have controversial. The sputum elastase activity correlated with the 24-h sputum recurrent pulmonary infections eventually lead to airway volume and the number of bronchiectatic lung lobes. radicals. is that the individual becomes antitrysin deficiency may cause emphysema alone. the underlying cause may be very difficult to oxide synthetase. in patients with Annals of Thoracic Medicine .3. [3] Neutrophils are thought to play a central role in the Immotile Cilia syndrome/Kartagener’s syndrome pathogenesis of tissue damage that occurs in bronchiectasis.246.[48] The common feature among all the conditions that A number of inflammatory mediators are involved in the lead to bronchiectasis.[58] When present colectomy. some patients with Endogenous nitric oxide production is involved in the inflammatory bowel disease. thereby reducing its uncommon. [56] It also affects the phagocytic and the Pulmonary involvement in inflammatory bowel disease is complement-fixing activity of IgG. inflammatory response and progressive lung damage. increased airway secretion. June 2006 43 . that the clinical expression of a α1­ inflammation. that bronchiectasis in in excess. have Kartagener’s syndrome. such as elastase and superoxide bronchiectasis occurs before the onset of Rheumatoid arthritis.[42. immotile cilia The progressive nature of bronchiectasis is thought to result syndrome can lead to bronchiectasis.[39-40] The combination of rheumatoid arthritis persistently high levels of elastase in their sputum. not all patients are persistently colonized with bacteria. damage of the ciliated epithelium and acceleration of airway may have increased susceptibility to respiratory infections inflammation. any damage to the airways that results bronchiectasis. Regardless emphysema with chronic bronchitis.[1-3] Approximately 50% of patients with immotile cilia syndrome. it was found that patients with In addition to promoting tissue damage. are intermittentHowever.: Pathogenesis. and bronchiectasis carries a poor prognosis. is that they either lead to alteration in recruitment and activation of neutrophils. or are associated with itself. immune response to the synovial membrane. [58] This correlated well bowel to the lung. in whom colonization with bacteria and Walker et al found that the incidence of bronchiectasis is 3. Shin et al suggested. occurring in 25% of cases with pulmonary involvement. The potential mechanisms for the reduction in exhaled nitric oxide in Pathogenesis of Bronchiectasis bronchiectasis.thoracicmedicine.[50] Neutrophil elastase is a serine proteinase that has that chronic suppurative infection leads to triggering an been implicated in the pathogenesis of bronchiectasis. found that the explain the occurrence of bronchiectasis.[1. June 10.[42-44] Interestingly. susceptible to bacterial colonization and infection. have airway opsonophagocytic function.Vol 1. are some patients. post-colectomy. In the study conducted by Swinson et al.49] This incidence of bronchiectasis in patients with rheumatoid reflects the degree of airway inflammation. those patients who to mucous gland hyperplasia.3] The sputum of these patients contains large patients with osteoarthritis. as compared to 0.[1.34. [4] damage.[1] The end result.[34] Solanki et al found that the amounts of serum proteins.[35] increased capillary permeability and exudation of serum proteins into the alveolar space.[49] Inherited as an autosomal recessive disease. consumption of nitric oxide by In spite of the numerous conditions that are associated with reaction with superoxide and the down regulation of nitric bronchiectasis.[46] This may highly cytotoxic compound. IP: 27. to form a and infection has a minor role in its pathogenesis.[37-38] In contrast.[59] It also reacts with superoxide anion. thus allowing bacterial adherence to the lung Inflammatory bowel disease epithelium.1% high levels of inflammatory markers in the sputum.45-46] It has been suggested. In addition. or emphysema with of the initiating event. there bronchiectasis.3] This may be mediated through its destructive effect on IgA.[59] Tsang et al. before or after the onset of that the sputum of patients with bronchiectasis has high levels Rheumatoid arthritis. [36-37] It has been suggested that.[55] This damage and bronchiectasis.[57] disease with bronchiectasis. renders the airways occur in those patients before the appearance of emphysema. It leads rheumatoid arthritis. develop bronchiectasis after the onset of rheumatoid arthritis. caused by rheumatoid arthritis itself or its treatment. it has been found Bronchiectasis can occur. may include poor nitric oxide diffusion through the diseased tissue. [51-54] In a study by Tsang and co-workers.[30] They also suggested that bronchiectasis may in loss of the mucociliary transport.2%. Infection leads to if they are exposed to recurrent respiratory tract infections. is due to an autoimmune process epithelium. have the worse 5­ evidence that neutrophil elastase promotes bacterial year survival compared to that of either diseases alone. has recently received considerable interest.

The most frequent abnormality was mucosal the diagnosis.[80. bronchiectasis. thus challenge test. In view of the large number of genetic Bruggen-Bogaarts et al. June 10. may be used as a screening test for primary ciliary Chest radiograph dyskinesia (PCD). Interleukin 6 (IL­ Pulmonary function studies may be normal in localized and 6). ß4). IP: 27. abnormalities seen on the chest radiograph. for the diagnosis of imaging technique of choice. followed by bronchomalacia. Although patients. there was a linear relationship appearance during fibro-optic bronchoscopy.Vol 1.[81] The initial test in adult hypercapnic respiratory failure and exacerbations. Pulmonary function tests Interleukin Iß (IL-1ß). bronchial wall thickening found on HRCT. concluded that.[2] Sweat chloride test In a study conducted in Saudi Arabia.140] Al-Shirawi. almost always excludes relevant bronchiectasis Bronchoscopy with a sensitivity of 87. Interleukin 8 (IL-8). A more recent study about the role of HRCT in findings were identified: mucosal inflammation. Chang et al. by interfering responsiveness. recurrent respiratory symptoms. et al .[86-88] Definitive 90% of symptomatic patients with bronchiectasis. Airway hyper-responsiveness is capacity.246. tram tracks.[65.[69-72] It helps to detect findings that are not outpatient setting. This focal pneumonitis.[65-66] It may patients with bronchiectasis. Interleukin 10 (IL-10). In addition. [73] Van der investigation of PCD. but can occur in other diseases as well.82] Using the above criteria. small bronchiectases and bronchomalacia. been shown that. airway disease (OAD) and those who did not. compared the role of chest mutations causing PCD.[2] such as CFTR mutation analysis. is a cost-effective method for diagnosing seen on plain chest radiographs.[80] However.[76-79] or any endobronchial lesion. patients with bronchiectasis. because it reduces motion artifacts. With more severe and diffuse disease.org on Friday. as well as clarifying the PCD. Chloride levels <40 mM with OAD. in the diagnosis of PCD. The airway abnormalities morphological findings on HRCT and disease activity. [60-62] These include. a normal sweat chloride test hyper-rectivity has a significant role in the management of cannot rule out cystic fibrosis and further tests are required. June 2006 . between 30-69% of frequently seen in patients with bronchiectasis. have evidence of airway hyper contribute to the pathogenesis of bronchiectasis.[60-64] The combination obstructive and restrictive abnormalities.[91] Five major airway X-ray. etiology and treatment of bronchiectasis bronchiectasis. correlated with the same lobe well as the degree of pulmonary function impairment. who have OAD.[80.thoracicmedicine.84] Diagnostic Testing Tests for primary ciliary dyskinesia Measurement of nitric oxide levels in exhaled breath condensate. Similarly. Tumour necrosis factor α (TNF-α) and Leukotriene ß4 (LT­ mild bronchiectasis. from 40-60 mM are borderline and mentioned above. airway obstruction and bronchial hyper­ levels >60 mM are abnormal. [74] They found that a normal chest radiograph. acts synergistically to induce hyperinflation and reduced carbon monoxide diffusing airway inflammation. Those patients usually present with higher prevalence of pseudomonas colonization. inflammation. to determine High-resolution CT (HRCT) of the chest has become the the most cost-effective method of biopsy. requires measurement of ciliary beat include hyperinflation. bronchoscopy is finding of small-airway abnormalities were associated with usually indicated to exclude the possibility of foreign body. is a significant determinant of airflow obstruction. with a high degree of accuracy.[90] for bronchiectasis.3 several studies have shown a link between the and 18. It has abnormality. using high-speed digital video photography.[85] Levels below 250 ppb are suggestive of Routine chest radiographs are abnormal in approximately PCD.[68] usually requires a biopsy taken from nasal or tracheal High-resolution CT scan epithelium. increased linear markings. as evident by histamine or methacholine with mucous clearance mechanism of the lung. Spiral CT scan may show subtle brushing is a very cost-effective way for the initial changes. Issue 1. genetic testing is not a very useful radiography and high-resolution CT Scan in the screening diagnostic tool for PCD. ring shadows and atelectasis. have until early adulthood.66] promoting bacterial colonization. There are multiple case reports 44 Annals of Thoracic Medicine .83. responsiveness play role in the pathogenesis of more than 90% of patients with cystic fibrosis will have bronchiectasis and treatment modalities that reduce airway abnormal test.8% of children.[80] However.[4] Evidence of of these inflammatory mediators. frequency.[89] They concluded that nasal biopsy collected in the bronchiectasis. Friedman et al performed a study. in patients with between the severity of bronchiectasis at HRCT and bronchiectasis. is the Sweat chloride test. In addition. may also be seen. that patients with bronchiectasis. They concluded to describe the bronchoscopic airway appearance in children that HRCT is rarely needed in the presence of a normal chest with non-cystic fibrosis bronchiectasis. while the In patients with localized bronchiectasis. combination of bronchiolectases may not be visible on chest X-ray and even bronchomalacia and obliterative-like lesions and no conventional CT scan and that HRCT is required to confirm abnormalities. as present on bronchoscopy. occurring in 58.[67] It was found there are few patients in whom the diagnosis is not made. MacCormick and coworker found that nasal findings from chest X-rays.[75] In addition. obliterative-like lesion. [Downloaded free from http://www. [67] As are considered normal.8% and no further investigation is There are few published data regarding the airway usually necessary. conducted a retrospective study.34. on the high resolution CT scan of the chest. TNF-α leads to the expression of chemo-attractants and pulmonary function tests may show obstructive or combined IL-8 leads to neutrophil degranulation. respectively. with about made between bronchiectatic patients who had obstructive 70% of cases diagnosed by the age of one year. 2016. this was statistically insignificant. a comparison was Cystic fibrosis is usually diagnosed early in life.[4] Findings testing for PCD.: Pathogenesis. presenting with clinical features suggestive of cystic there was a trend towards increased mortality in patients fibrosis. increase sputum production.

IgE)/ IgG subclasses drainage. in addition to the use of mucolytics. has not been could not find any randomized controlled trials addressing described.108] The reported before the age of 14 years.[101] Infection minimal mortality and morbidity. cepacia.5% of patients.[95.104-107] Operative the presence of varicose or cystic bronchiectasis. cepacia this issue. with bronchiectasis. is a matter of debate.[5] lung infection with pseudomonas aeruginosa.108] In a study conducted in Saudi Arabia to assess Not all patients with bronchiectasis who are colonized with the results of surgery for unilateral bronchiectasis.110-112] pseudomonas aeruginosa antibodies. regarding whether surgical treatment is superior to other non­ colonized with B.246. with improvement in 27. In the study by Angrill treatment. resulting in antigen­ uncontrolled hemorrhage and for the removal of damaged antibody immune complex. that compared surgical mechanical ventilation. cepacia. chest percussion. Patients used to be selected for surgery. in the differentiation between colonization and infection. followed by pseudomonas Spp (26%) and Streptococcus.140] Al-Shirawi.[97] morbidity ranges between 14 to 53%. transmission of the micro-organism. non-perfused) in patients with bronchiectasis. if life threatening complications occur such as different antigens of the pseudomonas. Ashour et pseudomonas. aeruginosa have worse quality of life. [104] Even those who survive. cepacia in non-cystic fibrosis individuals.107. correlates with clinical parameters in patients with hemodynamic-based classification. immuno-compromised patients and those requiring In a systematic review of the studies.34. A infection. It has been found that patients who have chronic similar to the numbers reported by others. Ho et al found that isolation of pseudomonas morphological and functional (perfused vs. if there is obstructing tumor or correlate with poor prognosis. Ledson et al. [Downloaded free from http://www.[109] The number of patients cured by the operation important. and coworkers. A majority of patients (more infected with P. Risk factors Surgical resection is considered one of the therapeutic options. June 10. presence of antibodies. for bacterial colonization. IgA. in different series.[97] The most usually have poor quality of life and incapacitating commonly found microorganism was hemophilus influenza symptoms. or immune-deficiency. pneumonia (12%). 2016.114] with Burkholderia cepacia is well known in patients with cystic fibrosis. etiology and treatment of bronchiectasis of complete resolution of bronchiectasis. The author postulated foreign body via fibro-optic bronchoscopy.[5. forced exhalation and controlled • Sweat chloride test • Rheumatoid factor cough. that aid the patient to remove respiratory • Complete and differential blood count secretions. the lower respiratory tract of patients with bronchiectasis. as a result of advances in therapy.106] (55%).: Pathogenesis. IP: 27. include diagnosis of bronchiectasis for the treatment of bronchiectasis. Pseudomonas aeruginosa morphological classification (cystic vs. was associated with high features.Vol 1. develop antibodies induced by the infection. Caballero et al studied the role of anti­ Mycobacterium avium complex. non-cystic).org on Friday. in patients with bronchiectasis.[5] microorganisms are responsible for the progressive tissue Before the development of surgical resection and antibiotic damage that occurs in bronchiectasis. they when medical treatment fails. al found no operative mortality and 15% operative Differentiation between colonization and infection is morbidity.96] These improved tremendously. inhaled broncho­ • Aspergillus precipitins dilators and corticosteroids. Bronchopulmonary hygiene • Chest X-ray Bronchopulmonary hygiene consists of different maneuvers • HRCT of chest and drugs. the mortality of bronchiectasis was as high as 49%. Microbiology of Bronchiectasis Treatment of Bronchiectasis In contrast to healthy non-smokers. based on at 75% sensitivity and specificity. such as postural • Immunoglobulin level (IgG. greater than 73%). June 2006 45 .[4] • Alpha-1 antitrypsin level • Sinus CT scan Trials have found that chest physical therapy improved • Sputum bacterial.114] Surgery is sputum volume and worse lung function (FEV 1/FVC performed on patients who have localized areas of cystic and <60%). differentiated between the two groups. Table 3: Diagnostic testing for bronchiectasis. This was implications. or inflammation. [115] Therefore.[99] They found that the The ideal classification for bronchiectasis. [92-94] Other that chronic colonization with B.106.6%.[103] The mother did not have evidence surgical therapy for patients. is proposed by some investigators. with extent of bronchiectasis and worse lung function.[102] However. [96. the incidence of bronchial colonization with in a followup of 3 to 6 years. In contrast. develop infection due to this microorganism. is frequently colonized with The life expectancy of patients with bronchiectasis has potentially pathogenic micro-organisms (PPMs). et al . Thirty percent (30%) of Surgery isolates were found to be resistant to antibiotics.[4. However. resulted from direct diagnostic workup is outlined in Table 3. because of therapeutic and prognostic was 72. PPMs was 64%. after removal of the of cystic fibrosis. as measured by sputum production and Annals of Thoracic Medicine . [5. mycobacterial.[104. these Surgery in patients with bronchiectasis is usually indicated antibodies don’t protect against the infection. It includes physical therapy. which is based both on bronchiectasis. from the siblings to their mother. fungal culture and sensitivity pulmonary clearance. This resulted in lung damage and bronchiectasis.thoracicmedicine. IgM. the authors with B.[113.[98] The antibodies react with foreign body. that leads to chronic lung due to multi-drug resistant tuberculosis. no conclusion could be obtained bronchieotasis in a mother of two siblings with cystic fibrosis. Issue 1.[100] Wilson and co-workers also found that patients non-perfused bronchiectasis.[113. reported a case of B. chronic colonization versus non-surgical treatment of bronchiectasis.5%. FEV1 <80% of predicted value and operative mortality rates are 1-8. achieved excellent results after surgery.105.

is insufficient to draw any conclusion treatment. in 10 patients majority. [135-136] In a non-randomized study. there is no randomized controlled trial that examined their This was demonstrated in several lung diseases such as effect in such patients. to show 1000 mcg/day. has been reported to produce volume and purulence.[3. a systematic review concluded that the evidence albumin increased again. has been studied in a large randomized Antibiotics have several effects on the lung. In both studies. a recent systematic randomized study had examined the role of oral steroids in review of chest physical therapy in patients with acute or stable bronchiectasis. concluded that there is insufficient evidence be beneficial in patients with bronchiectasis.[139] Recently. Antibiotics have been shown to reduce the exacerbations. et al . oral steroids may bronchiectasis. responsiveness in patients with bronchial asthma and bronchiectasis. Their aim is to make it acute infective exacerbations of bronchiectasis. limitation may even become worse. Kelly et al studied the effect of 3 weeks Bronchiectasis is frequently treated with long-acting β2 treatment with amoxycillin.[123] Tsang et al studied the use of Fluticasone high dose of antibiotics over longer periods of time. June 10. who have chronic purulent sputum. is unknown. in the treatment in significant fall in sputum elastase activity. Bromhexine courses of high dose antibiotics result in reduction in sputum treatment for more than 7 days. in patients with bronchiectasis. resulted in a decrease in airway with bronchiectasis. bronchiectasis.[139] They found that the effectiveness of long-acting bronchodilators. treated with antibiotics for 14 days. etiology and treatment of bronchiectasis radioisotope clearance.246.[119] The use of aerosolized course of antibiotics in the management of bronchiectasis. The first As Leukotriene receptor antagonists have an effect on includes reduction in airway inflammation and the other is neutrophil-mediated inflammation. [124] They showed that the use of inhaled the purulent sputum retained rapidly in these patients.[127] bronchial asthma.org on Friday. IL-8 and LT-ß4).[138] The levels returned back to pre­ reduction in sputum volume and improvement of the FEV1.34. no change in pulmonary function. Although some trials have shown some albumin level. in patients bronchopulmonary aspergillosis (ABPA).[135] ventilatory defect and during exacerbation. is reducing airway The role of long-acting β2-agonists hyper-responsiveness. [3] In a study of 15 patients with bronchiectasis. the level of elastase and benefit.Vol 1. the use of rhDNase is not recommended in neutrophil elastase and the degree of protein transudation. as well as of bronchiectasis. as well as accelerate the decline in FEV1. for the patients to clear their sputum. they demonstrated that antibiotic treatment resulted effectiveness of short acting beta-2 agonists. Its use in patients with bronchiectasis. However. Two bronchiectasis postulated mechanisms have been proposed. in the prolonged antibiotic treatment (over 4 months). after stopping the antibiotics. in the secretions. Elborn and coworkers treated with two weeks antibiotics for acute exacerbation.: Pathogenesis. patients with purulent sputum needed bronchiectasis. is approved for role of long-term antibiotic use.[120] microbial load within the sputum and decrease the level of Therefore. in patients treatment with amoxycillin.[130] However.[3] patients with Cystic fibrosis. after that treatment with inhaled corticosteroid. Issue 1. in patients with controlled trial and has been found to increase the rate of bronchiectasis.[126] Further studies are needed to establish. during acute the patient.[2] Macrolides have several anti­ may have a role in the treatment of bronchiectasis.117] However. Stockley The use of inhaled bronchodilators and corticosteroids et al demonstrated a reduction in neutrophil elastase activity Bronchiectasis is usually associated with an obstructive and sputum albumin level. on the degree of airway agonists. inflammatory effects.[118] Effects of antibiotics in bronchiectasis Mucolytics are used in patients with bronchiectasis to assist Antibiotics have been used successfully in the treatment of in the bronchpulmonary clearance. as well as clinical improvement of some beneficial effect in sputum clearance. after stopping the antibiotic currently available. corticosteroid was associated with a reduction in the sputum inflammatory markers (IL-1 ß. 2016.140] Al-Shirawi. hyper-responsiveness. Another effect of antibiotics in bronchiectatic patients.[116. Hill et al regarding the use of short-acting beta-2 agonist. in addition to their anti-microbial action. in bronchiectatic patients. the airflow limitation is poorly reversible.thoracicmedicine. having allergic to support the routine use of chest physiotherapy. treatment level. bronchiectasis.132-134] Short easy. diffuse panbronchiolitis (DPB) and cystic fibrosis (CF).[125] They found chemotactic activity (NCA) and elastase activity (EA). it was thought that they related to clearing of sepsis. There is no randomized controlled trial to investigate responsiveness.[124-127] No large cystic fibrosis.[131] with bronchiectasis.[3. June 2006 . the recombinant human DNase (rhDNase).[142-155] Oral steroids for bronchiectasis Studies have shown small benefits from inhaled Inhaled antibiotics have been used extensively in patients with Corticosteroids in patients with bronchiectasis.[136] are no randomized controlled trials that assessed the Similarly. Hill et al studied the effects of patients with bronchiectasis may also have asthma.[137] Again. resulted in one week of treatment. for four weeks in patients with clinical improvement. [Downloaded free from http://www. after stopping the antibiotic. the studied the effect of six-week treatment with beclomethasone authors were able to demonstrate a fall in sputum neutrophil 1500 mcg/day. [121] Although many from purulent to mucoid.141] The mechanism of action of macrolides The use of Leukotriene receptor antagonists for in reducing airway responsiveness. several studies have found if they have a role in the treatment of patients with that macrolide antibiotics can reduce airway hyper­ bronchiectasis. in demonstrated that.140. in 12 of the 15 patients studied. is less well established. but to lesser extent in patients with non-cystic 46 Annals of Thoracic Medicine .133] In contrast to the well established role of short exacerbation of bronchiectasis. on 12 patients with bronchiectasis.[2.[122] There with bronchiectasis. the airflow These changes were associated with change of the sputum. but there was Another study by Ip et al. IP: 27.

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