Ce diaporama a bien été signalé.
Nous utilisons votre profil LinkedIn et vos données d’activité pour vous proposer des publicités personnalisées et pertinentes. Vous pouvez changer vos préférences de publicités à tout moment.


8 359 vues

Publié le

These lecture notes were prepared by Dr. Hamdi Turkey- Pulmonologist- Department of internal medicine - Taiz university

Do Not Forget To Visit Our Pages On Facebook on the following Links:

Publié dans : Santé & Médecine
  • do you have slides regarding abt lung cancer? it would b lovely if u can upload it thanks in advance
    Voulez-vous vraiment ?  Oui  Non
    Votre message apparaîtra ici


  1. 1. Bronchiectasis HamdiAl-Turkey-chest physician
  2. 2. Objectives To know diseases that are associated with BR. To have a general idea about the major clinical features. To know how to treat various causes of bronchiectasis. Identify key differences in the treatment between BR and CF.
  3. 3. Definition Bronchiectasis (broncos, airways; ectasia, dilatation) is a morphological term used to describe abnormal irreversibly dilated and often thick- walled bronchi. This is an anatomic definition and is thought to have evolved from Laennec’s original description in 1819 of ectatic bronchi in pathological specimens. Bronchiectasis represents the end stage of a variety of pathologic processes that cause destruction of the bronchial wall and its surrounding supporting tissues.
  4. 4. History Described initially in the early 19 th century By Laënnec in patients with chronic suppurative phlegm. “This affection of the bronchia is always produced by chronic catarrh, or by some other disease attended by long, violent, and often repeated fits of coughing”. R.T.H. Laënnec.
  5. 5. Sir William Osler “Dilation of the bronchi occurs … As a congenital defect or anomaly. Such cases are extremely rare… In connection with inflammation of the bronchi, particularly when this leads to weakness of the walls with the accumulation of secretion.”
  6. 6. Prevalence Bronchiectasis was a common disabling and fatal condition in the pre-antibiotic era. It remains an important cause of suppurative lung disease in the developing world. More recently, the declining incidence of this disease in the developed world has led to repeated suggestions that it be considered an orphan disease. In the United States, the prevalence has recently been estimated to be 52 per 100,000. A slight female preponderance has been suggested
  7. 7. Pathology bronchial dilatation is associated with destructive and inflammatory changes in the walls of medium-sized airways, often at the level of segmental or subsegmental bronchi Airway inflammation is primarily mediated by neutrophils and results in up-regulation of enzymes such as elastase and matrix metalloproteinases The normal structural components of the wall, including cartilage, muscle, and elastic tissue, are destroyed and may be replaced by fibrous tissue.
  8. 8. Pathology The dilated airways frequently contain pools of thick, purulent material, while more peripheral airways are often occluded by secretions or obliterated and replaced by fibrous tissue Additional microscopic features include bronchial and peribronchial inflammation and fibrosis, ulceration of the bronchial wall, squamous metaplasia, and mucous gland hyperplasia The parenchyma normally supplied by the affected airways is abnormal, containing varying combinations of fibrosis, emphysema, bronchopneumonia, and atelectasis
  9. 9. Pathology As a result of inflammation, vascularity of the bronchial wall increases, with associated enlargement of the bronchial arteries and anastomoses between bronchial & pulmonary arterial circulations
  10. 10. Epithelial injury Mucus hypersecrtion Reduced mucociliary clearance Plugging of the airways InflammationChronic bronchial infection Airway damage Bronchiectasis Mucus secretogogus Ciliotoxin Reactive oxygen species Proteinase enzymes IL-8 TNF-alpha LTB4 Reactive oxygen species Elastase MMPs
  11. 11. Post infective • Severe pneumonia • Tuberculosis • Pertussis • Measles Impaired mucociliary clearance • CF • Primary ciliary dyskinesia • Young’s syndrome Immunodeficiency • Common variable immunodeficiency • Specific polysaccharide antibody deficiency Secondary immunodeficiency,eg, malignancy(chronic lymphocytic leukemia)or human immunodeficiency virus infection Exaggerated immune response • Allergic broncho pulmonary aspergillosis • Graft versus host disease • Inflammatory bowel disease (ulcerative colitis and Crohn’s disease) . Congenital abnormalities of the bronchial wall • Mounier-Kuhn syndrome • Williams-Campbellsyndrome • Marfan syndrome Inflammatory pneumonitis • Aspiration of gastric contents • Smoke inhalation Fibrosis(traction bronchiectasis) • Sarcoidosis • Idiopathic pulmonary fibrosis Mechanical obstruction • Foreign body • Tumor • Extrinsic compression (eg,lymph node) Miscellaneous conditions • Primary Mycobacterium avium complex infection(“Lady Windermere syndrome”) Connective tissue diseases • rheumatoid arthritis • systemic lupus erythematosus • Sjo¨gren syndrome Pulmonary sequestration Yellow nail syndrome Infertility(primary ciliary dyskinesia,cystic fibrosis,Young syndrome) Diffuse pan bronchiolitis a1-Antitrypsin deficiency
  12. 12. What is the current relevance of previous severe lower respiratory tract infections to patients with bronchiectasis? • Bronchiectasis may be the sequela of a variety of necrotizing infections that are either inadequately treated or not treated at all. • Primary infection (ie, in the absence of intrinsic defects or noninfectious extrinsic insults) was a particularly common cause of bronchiectasis in developed countries prior to the widespread use of antibiotics and it remains important in developing countries, where antibiotics are used inconsistently.
  13. 13. Klebsiella species Staphylococcus aureus Mycobacterium tuberculosis Mycoplasma pneumoniae Nontuberculous mycobacteria Measles virus Pertussis virus Influenza virus Herpes simplex virus Certain types of adenovirus Typical infectious agents that have been shown to cause Bx
  14. 14. Focal post-obstructive bronchiectasis may occur in a number of clinical settings (eg, endobronchial tumors, broncholithiasis, bronchial stenosis from infections, encroachment of hilar lymph nodes, foreign body aspiration). Right-middle lobe syndrome is a specific type of bronchial obstruction that may result in bronchiectasis. It results from an abnormal angulation of the lobar bronchus at its origin, predisposing it to obstruction, subsequent infection, and development of bronchiectasis. Bronchial obstruction
  15. 15. In adults, foreign body aspiration often takes place in the setting of altered mental status and involves unchewed food. Patients may also aspirate chewed materials from the stomach, including food, peptic acid, and microorganisms. After aspiration, a post-obstructive pneumonia may occur, with subsequent development of focal bronchiectasis. Bronchiectasis may also develop in the setting of chronic aspiration. Further recognized is that a history of gastroesophageal reflux is a risk factor for aspiration and that the organism Helicobacter pylori may play a role in the development of bronchiectasis in this group of patients. Aspiration
  16. 16. Immunodeficiency states may be congenital or acquired. The most common congenital conditions (albeit rare) involve B-lymphocyte functions. Hypogammaglobulinemia in these cases may take one of the following forms : • Immunoglobulin G (IgG) subclass deficiency • X-linked agammaglobulinemia • Immunoglobulin A (IgA) deficiency • Immunoglobulin M (IgM) deficiency • Immunoglobulin E (IgE) deficiency Patients with hypogammaglobulinemia usually present in childhood with repeated sinus or pulmonary infections, although the disorder has been diagnosed in adults who did not have a history of repeated infections. Establishing the diagnosis is important because gammaglobulin replacement may reduce the number of infections and resultant lung injury. Immune deficiency and Bx
  17. 17. ►The possibility of underlying immune deficiency, particularly antibody deficiency, should be considered in all children and adults with bronchiectasis. ►Serious, persistent or recurrent infections, particularly involving multiple sites, or infections with opportunist organisms should raise the suspicion of immune deficiency. ►The possibility of symptomatic or clinically silent bronchiectasis should be considered as a potential complication in all patients with immune deficiency, particularly primary antibody deficiency. ►In patients with immune deficiency and patients with bronchiectasis, features in the history or clinical examination which may support the coexistence of both conditions should be considered and adequately assessed. ►Humoral immunodeficiency syndromes involving deficiencies of IgG, IgM, and IgA can cause recurrent suppurative sinopulmonary infections and BR. Immune deficiency and Bx
  18. 18. CF is a multisystem disorder that affects the chloride transport system in exocrine tissues, primarily secondary to a defect in the CF transmembrane regulator (CFTR) protein. CF and its variants are the most common cause of bronchiectasis in the United States and other industrialized nations. CF is an autosomal recessive disease affecting approximately 1 in 2,500 whites and 1 in 17,000 blacks in the United States. The major pulmonary finding in CF is bronchiectasis, which is an almost universal feature of this disease. It may be the sole feature of CF in adults or those with genetic variations of the disease. Bronchiectasis associated with CF is believed to occur secondary to mucous plugging of proximal airways and chronic pulmonary infection, especially with mucoid P aeruginosa. Cystic Fibrosis (CF)
  19. 19. One of these plus one of these ≥1 typical phenotypic features of CF Elevated sweat chloride test on 2 occasions Sibling with CF 2 identified CFTR mutations Positive newborn screening test (IRT) Abnormal nasal potential difference (at research centers) Dr Hamdi Turkey Diagnostic criteria for CF
  20. 20. Mutant CFTR gene in CF
  21. 21. Autosomal recessive inheritance
  22. 22. Julia and CF
  23. 23. Young syndrome is clinically similar to CF and may represent a genetic variant of CF. It is most often observed in middle-aged men in North America and is a leading cause of male infertility. Patients with Young syndrome have bronchiectasis (often predominant in the lower lobes), sinusitis, and obstructive azoospermia. However, they do not display the other findings of CF. The pathogenesis of bronchiectasis in these patients is believed to be similar to that of bronchiectasis in CF. The criterion standard for diagnosis of Young syndrome is electron microscopic analysis of the structure of the cilia. Young syndrome
  24. 24. Primary ciliary dyskinesia is a group of inherited disorders that may affect 1 in 15,000-30,000 population. It is manifested by immotile or dyskinetic cilia and/or sperm. PCD is a genetically heterogeneous disorder affecting motile cilia which are made up of approximately 250 proteins. Around 90%of individuals with PCD have ultrastructural defects affecting protein(s) in the outer and/or inner dynein arms which give cilia their motility, with roughly 38% of these defects caused by mutations on two genes, DNAI1 and DNAH5, both of which code for proteins found in the ciliary outer dynein arm. • The main consequence of impaired ciliary function is reduced or absent mucus clearance from the lungs, and susceptibility to chronic recurrent respiratory infections, including sinusitis, bronchitis, pneumonia, and otitis media. Progressive damage to the respiratory system is common, including progressive bronchiectasis beginning in early childhood, and sinus disease (sometimes becoming severe in adults) • diagnosis is often missed early in life despite the characteristic signs and symptoms. In males, immotility of sperm can lead to infertility Primary ciliary dyskinesia
  25. 25. Schematic diagram of the eukaryotic cilium. Cross-section illustrates the 9 + 2 configuration of nine peripheral microtubular doublets surrounding a central pair microtubule complex. The expanded view of a microtubular doublet schematically depicts crosssections of the tubulin protofilaments including those shared by the A and B tubules. The dynein arms in the expanded view are rendered to schematically depict several light, intermediate, and heavy chains comprising each of these structures. Although the outer arm exhibits a specific distribution of dyneins, being uniformly composed of three heavy, two intermediate and at least eight light chains, the distribution of dyneins in the inner arm is thought to be more variable.
  26. 26. Electron micrographs of nasal cilia from patients with primary ciliary dyskinesia (PCD) illustrating dynein defects. Far left panel illustrates ultrastructure of a normal cilium from nasal epithelium of a healthy, clinically unaffected subject. The adjacent panels from three different patients with PCD illustrate defects in both dynein arms, isolated defects of outer dynein arms only, and isolated defects of inner dynein arms only.
  27. 27. Kartagener syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive condition characterized by abnormal ciliary structure and/or function leading impaired mucociliary clearance. situs inversus chronic sinusitis and/or nasal polyposis bronchiectasis Kartagener syndrome is characterised by the clinical triad of: Kartegner syndrome
  28. 28. Kartegner syndrome
  29. 29. • Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to inhaled Aspergillus antigen that is characterized by bronchospasm, bronchiectasis, and immunologic evidence of a reaction to Aspergillus species. • ABPA should be suspected in patients with a productive cough who also have a long history of asthma-type symptoms that do not respond to conventional therapy. • Bronchiectasis is believed to be secondary to airway plugging by viscid secretions containing hyphae of Aspergillus species. The resulting bronchiectasis is thin-walled and affects the central and medium-sized airways. Allergic bronchopulmonary aspergillosis
  30. 30. Dr Hamdi Turkey Diagnostic criteria for ABPA
  31. 31. Diagnostic criteria for ABPA
  32. 32. Bronchiectasis can result from a variety of congenital anatomic defects : Bronchopulmonary sequestration is a congenital abnormality classified as either intralobar or extralobar and results in chronic lower respiratory tract infections that lead to bronchiectasis. Williams-Campbell syndrome (congenital cartilage deficiency) is the absence of cartilage from lobar to first- to second-generation segmental airways that results in extensive peripheral bronchiectasis. Mounier-Kuhn syndrome (tracheobronchomegaly) is a rare disorder characterized by dilation of the trachea and segmental bronchi (central bronchiectasis). Swyer-James syndrome (unilateral hyperlucent lung) likely is a developmental disturbance that leads to unilateral bronchiolitis, hyperinflation, and, in some cases, bronchiectasis. Yellow-nail syndrome is rare. It results in exudative pleural effusions. Congenital anatomical defects
  33. 33. This patient had a 20-year history of severe lymphedema of her legs; thick, ridged, yellowish, hypercurved thumbnails (top right); similarly affected, yellow-green to brown toenails (bottom right); and bilateral, chylous pleural effusions. A sample of her chylous pleural fluid is shown to the left of the radiograph. This syndrome of yellow nails, lymphedema, and pleural effusions (usually serous) presumably results from defective lymphatic drainage. Women are affected almost twice as much as men, and prolonged survival is the rule. Yellow nail syndrome
  34. 34. Clinical features
  35. 35. Symptoms of bronchiectasis Cough Chronic productive cough is prominent,occurring in up to 98% of patients. Sputum is typically produced on a daily basis in greater than 70% of patients, Some patients produce sputum only with acute upper respiratory tract infections, but otherwise they have quiescent disease. Sputum is typically mucoid and relatively odorless. During infectious exacerbations, however, sputum becomes purulent and may develop an offensive odor. In the past, total daily sputum amount has been used to characterize the severity of bronchiectasis, with less than 10 mL defined as mild bronchiectasis, 10-150 mL defined as moderate bronchiectasis, and greater than 150 mL defined as severe bronchiectasis. dry bronchiectasis manifests as episodic hemoptysis with little-to-no sputum production. Dry bronchiectasis is usually a sequela of tuberculosis. Hemoptysis Hemoptysis occurs in 56-92% of patients with bronchiectasis. Hemoptysis is more commonly observed in dry bronchiectasis. Hemoptysis is generally mild and manifested by blood flecks in the patient's usual purulent sputum. Bleeding usually originates from dilated bronchial arteries, which contain blood at systemic pressures. Therefore, massive hemoptysis may occur but is rarely a cause of death. Dyspnea Dyspnea may occur in as many as 72% of patients; a 2006 review reported a rate of 62%. Dyspnea typically occurs in patients with extensive bronchiectasis observed on chest radiographs.
  36. 36. Symptoms of bronchiectasis Wheezing Wheezing is commonly reported and may be due to airflow obstruction following destruction of the bronchial tree. Similar to dyspnea, it may also be secondary to concomitant conditions such as asthma. Chest pain Pleuritic chest pain is an intermittent finding, occurring in 19-46% of patients. It is most commonly secondary to chronic coughing but also occurs in the setting of acute exacerbation. Fatigue Fatigue is commonly reported (73% of patients). Weight loss often occurs in patients with severe bronchiectasis. This is believed to be secondary to increased caloric requirements associated with the increased work of coughing and clearing secretions. Fever Fever may occur in the setting of acute infectious exacerbations. Urinary incontinence Urinary incontinence occurs more frequently in women with bronchiectasis versus age-matched controls (47% vs 12%).The etiology of this is unclear.
  37. 37. Physical signs Wheeze Scattered wheezing may be heard in approximately one third of patients; wheezing may be due to airflow obstruction from secretions, destruction of the bronchial tree leading to airway collapsibility, or a concomitant condition. Crackles Crackles and rhonchi are often observed in association with active infections and acute exacerbations Clubbing Digital clubbing is an inconsistent finding in approximately 2-3% of patients ; it is more frequent in patients with moderate-to-severe bronchiectasis Signs of cor pulmonale Right-sided heart failure may be observed, including peripheral edema, hepatomegaly, and hypoxia
  38. 38. Bronchiectasis as a result of infection generally involves the lower lobes, the right-middle lobe, and the lingula Right-middle lobe involvement alone suggests right-middle lobe syndrome, an anatomic dysfunction, or a neoplastic cause with secondary mechanical obstruction Bronchiectasis caused by cystic fibrosis (CF), Mycobacterium tuberculosis infection , or chronic fungal infections tends to affect the upper lobes, although this is not universal in CF Allergic bronchopulmonary aspergillosis (ABPA) also affects the upper lobes but usually involves the central bronchi, whereas most other forms of bronchiectasis involve distal bronchial segments The anatomical distribution of bronchiectasis may be important in helping diagnose any associated condition or cause of bronchiectasis
  39. 39. Investigations
  40. 40. sputum analysis A sputum analysis may reinforce the diagnosis of bronchiectasis and add significant information regarding potential etiologies. Once sputum is allowed to settle, the examination may reveal Dittrich plugs, small white or yellow concretions. A Gram stain and culture result may reveal evidence of microorganisms, including mucoid Pseudomonas species and Escherichia coli, which suggest CF but are not diagnostic. Chronic bronchial infection with nonmucoid Pseudomonas aeruginosa is becoming much more common in patients with non-CF bronchiectasis. The presence of eosinophils and golden plugs containing hyphae suggests Aspergillus species, although this finding alone is not diagnostic of ABPA. Perform a smear and culture of sputum for mycobacteria and fungi. Atypical mycobacterial infection is a common cause of bronchiectasis in the older population, especially in those with underlying structural lung disease. Consider suppurative lung disease when you encounter three layered sputum (a purulent sediment, clear middle liquid and a top foamy layer) with foul smell.
  41. 41. Laboratory investigations CBC The CBC is often abnormal in patients with bronchiectasis. Typical findings are nonspecific and include anemia and an elevated white blood cell count with an increased percentage of neutrophils. An increased percentage of eosinophils is one criterion for ABPA. Alternatively, polycythemia secondary to chronic hypoxia may be observed in advanced cases. immunoglobulin level including IgG subclasses, IgM, and IgA, are useful to exclude hypogammaglobulinemia. Note, however, that on rare occasions, bronchiectasis may be seen in patients with antibody production deficiency but normal to low- normal IgG levels. In situations such as these, evaluating antibody response to Haemophilus influenzae and pneumococcal vaccines may be useful. Alpha1- Antitrypsin Quantitative serum alpha1-antitrypsin (AAT) levels are used to rule out AAT deficiency. In addition to a suggestive family history, clinical features of emphysema that suggest the possibility of AAT deficiency and the need for serum testing include onset at an early age (45 y or less) and the absence of a recognized risk factor (eg, smoking, occupational dust exposure). Sweat test Pilocarpine iontophoresis (sweat test) was the criterion standard test to evaluate for CF. However, genetic analysis has now become standard and may be performed to look for evidence of mutations consistent with CF and to look for potential variants, such as Young syndrome.
  42. 42. Lab. Investigations (2) Aspergillus Precipitins and Serum Total IgE levels Aspergillus precipitins and serum total IgE levels are important in making the diagnosis of ABPA. Diagnostic criteria for ABPA include a total serum IgE level greater than 1000 IU/mL or a greater than 2-fold rise from baseline. Autoimmune Screening Rheumatoid factor and/or other screening tests for autoimmune disease may be performed in the appropriate clinical setting. For example, an antinuclear antibody (ANA) assay may also be considered. Electron Microscopic Examination Perform electron microscopic examination of sperm and respiratory epithelium to observe for evidence of primary ciliary structural abnormalities and dyskinesia. These will be found in disorders such as primary ciliary dyskinesia. Spirometry Pulmonary function test results may be normal or abnormal. The most common abnormality is an obstructive airway defect, which may even be found in patients without a prior smoking history.
  43. 43. CXR Posterior-anterior and lateral chest radiographs should be obtained in all patients. Expected general findings include increased pulmonary markings, honeycombing, atelectasis, and pleural changes. Specific findings may include linear lucencies and parallel markings radiating from the hila (tram tracking) in cylindrical bronchiectasis, dilated bronchi in varicose bronchiectasis, and clustered cysts in cystic bronchiectasis. In the appropriate clinical setting, chest radiograph findings are occasionally sufficient for confirming the diagnosis of bronchiectasis.
  44. 44. HRCT of the chest CT scanning, particularly high-resolution CT (HRCT) scanning of the chest, has replaced bronchography as the defining modality of bronchiectasis. CT sensitivity and specificity reportedly are 84-97% and 82-99%, respectively, but may be higher at referral centers. The following are noteworthy aspects of CT findings in bronchiectasis: Cylindrical bronchiectasis has parallel tram track lines, or it may have a signet-ring appearance composed of a dilated bronchus cut in a horizontal section with an adjacent pulmonary artery representing the stone The diameter of the bronchus lumen is normally 1-1.5 times that of the adjacent vessel; a diameter greater than 1.5 times that of the adjacent vessel is suggestive of bronchiectasis Varicose bronchiectasis has irregular or beaded bronchi, with alternating areas of dilatation and constriction Cystic bronchiectasis has large cystic spaces and a honeycomb appearance; this contrasts with the blebs of emphysema, which have thinner walls and are not accompanied by proximal airway abnormalities
  45. 45. Cylindrical bronchiectasis. A, B, Examples from two patients. Airways parallel to the plane of section in the anterior segment of an upper lobe show changes of cylindrical bronchiectasis; bronchi are wider than normal and fail to taper as they proceed towards the lung periphery. Varicose bronchiectasis. Patient with allergic bronchopulmonary aspergillosis and cystic fibrosis. The bronchiectatic airways have a c o r r u g a t e d , o r b e a d e d , appearance. Cylindrical bronchiectasis Varicose bronchiectasis
  46. 46. Cystic bronchiectasis in the upper lobes in two patients. In such advanced disease, it is often impossible to distinguish between markedly dilated bronchi and cystic airspaces in destroyed lung. A, Etiology of the bronchiectasis was unknown in this patient. B, Patient had cystic fibrosis. Cystic bronchiectasis
  47. 47. Management
  48. 48. General approach and treatment of the specific underlying cause ►Identify and treat underlying cause to prevent disease progression. ►Maintain or improve pulmonary function. ►Reduce exacerbations. ►Improve quality of life by reducing daily symptoms and exacerbations. ►In children, achieve normal growth and development. ►Patients with primary or secondary immune deficiency should be under joint care with a clinical immunologist. ►Patients with CF should be referred to a CF specialist centre.
  49. 49. The following general measures are recommended: Smoking cessation Avoidance of second-hand smoke Adequate nutritional intake with supplementation, if necessary Immunizations for influenza and pneumococcal pneumonia. Confirmation of immunizations for measles, rubeola, and pertussis Oxygen therapy is reserved for patients who are hypoxemic with severe disease and end-stage complications, such as cor pulmonale. Supportive treatment
  50. 50. Physiotherapy: airway clearance techniques and exercise Good bronchial hygiene is paramount in the treatment of bronchiectasis, because of the tenacious sputum and defects in clearance of mucus in these patients. Postural drainage with percussion and vibration is used to loosen and mobilize secretions. Devices available to assist with mucus clearance include flutter devices, intrapulmonic percussive ventilation devices, and incentive spirometry.Although consistent benefits from these techniques are lacking and vary with patient motivation and knowledge, a review did report improvement in patients’ cough-related quality of life scores. Nebulization with concentrated (7%) sodium chloride solutions appears to be beneficial, particularly in patients with CF-related bronchiectasis. Mucolytics, such as acetylcysteine, are also often tried but do not appear to be universally beneficial. However, maintaining adequate general hydration, which may improve the viscidity of secretions, is important. Aerosolized recombinant DNase has been shown to benefit patients with CF. This enzyme breaks down DNA released by neutrophils, which accumulates in the airways in response to chronic bacterial infection. However, improvement has not been definitively shown in patients with bronchiectasis from other causes.
  51. 51. Chest physiotherapy (CPT) Chest physiotherapy (CPT) is a set of techniques that include percussion, vibration, and postural drainage. The purpose of CPT is to loosen respiratory secretions and move them into the central airways where they can be removed by coughing or suctioning. Removing secretions from the airway and not allowing them to accumulate reduces the risk for respiratory infections and atelectasis. CPT is often performed along with other therapies such as bronchodilators, antibiotics, mucolytic agents, and hydration. Combing these therapies helps reduce mucous production and increase airway clearance.
  52. 52. Chest percussion Percussion involves striking the skin over congested lung fields to dislodge secretions from the bronchial walls. It can be accomplished using cupped hands or commercial devices such as percussion cups. When you use your hands to percuss, hold your fingers and thumb together and flex them slightly to form a cup. When your cupped hand contacts the area to be percussed, the air is trapped against the chest, propelling vibrations through the chest wall to the secretions. When performing percussion, cover the area you are percussing with a towel or the patient’s gown to reduce any discomfort. Instruct the patient to breathe slowly and deeply and, in an alternating manner, flex and extend your wrists rapidly to percuss the chest. Percuss each area of the lung that is congested for 1 to 2 minutes or as prescribed. Avoid percussion over the breasts, sternum, spinal column, and kidneys.
  53. 53. Vibration Vibration is used after percussion or alternately with percussion to increase the turbulence of exhaled air and loosen secretions. To perform vibration, place your hands on the affected area either side by side or with one hand on top of the other and your fingers extended and together. While the patient exhales slowly through pursed lips or the nose, tense your hand and arm muscles and move the heel of your hand in a shaking manner to create vibrations through the patient’s chest. When the patient inhales, stop moving your hands. Perform vibration over the affected area during five exhalations, or as prescribed. After each vibration, instruct the patient to cough.
  54. 54. Postural drainage Postural drainage is performed to remove secretions by gravity from different areas of the lungs. To drain the affected areas, place the patient in a variety of positions to facilitate drainage by gravity. Not all positions are required for every patient and, depending on the patient’s illness or condition, some positions may be contraindicated. Postural drainage is commonly performed two or three times a day, often before meals and at bedtime. It is best to schedule it when the patient’s stomach is empty to avoid gastric reflux and vomiting. If the patient is receiving continuous tube feedings, stop the feeding and check gastric residual at least 30 minutes before performing postural drainage. Before starting postural drainage and during the procedure, evaluate the patient’s tolerance of the various positions. The patient usually remains in each position for 10 to 15 minutes. However, this time may be shorter initially and then gradually increased as the patient is better able to tolerate it. When performing postural drainage, first position the patient, then percuss and vibrate, then remove the secretions either by having the patient cough or by suctioning the patient’s airway.
  55. 55. Antibiotics • Antibiotics are given during the acute exacerbation ( empiric with amoxicillin 500 mg tid for 7-10 days or other antibiotics like ciproflxacin 500 mg bid for 7-10 days and other alternatives according to sputum culture •Patients with bronchiectasis are at increased risk of frequent infectious exacerbations. • Prevention of these Exacerbations is thought to be achieved by decreasing the “Bacterial Load” in the airways. •Several strategies have been tried to minimize the risk of these exacerbations.
  56. 56. Preventive antibiotic therapy • Daily oral antibiotic treatment, such as Ciprofloxacin 500 to 1500 mg/day, given in 2 to 3 divided doses, given for seven to 14 days of each month . • Erythromycin for 8 weeks A double-blind placebo-controlled study. To evaluate the effects of 8-week administration of low dose erythromycin ( Patients in the erythromycin group had significantly improved forced expiratory volume in one second, forced vital capacity and 24-h sputum volume after 8 weeks (p<0.05) • Daily or three times weekly use of azithromycin (250mg)
  57. 57. • Aerosolization of an antibiotic • Tobramycin at a dose of 300 mg BID every other month for six months in patients with CF had a 100-fold reduction in sputum Pseudomonas density, improved FEV1, and decreased hospitalizations compared to patients receiving placebo aerosol Preventive antibiotic therapy
  58. 58. IV antibiotics • Intermittent IV antibiotics should be reserved for patients with resistant organisms (such as Pseudomonas) or in preparation for major surgery, including resection of a bronchiectatic region of lung and other procedures during which pulmonary function may be compromised.

  59. 59. Pseudomonas • Almost impossible to eradicate in patients with bronchiectasis. • Patients have reduced quality of life indices, more extensive bronchiectasis on CT, and increased number of hospitalizations.
  60. 60. MAC • MAC is often harbored in damaged lung tissue and bronchiectatic airways. • ATS recommends treatment until the patient is culture negative for 12 months. • A combination of azithromycine/ clarithromycine + rafimpicin+ ethumbatol
  61. 61. ABPA • Oral Prednisone (0.5-1.0 mg/kg/day) is the cornerstone of therapy for patients with allergic bronchopulmonary aspergillosis. • Patients may also benefit from additional therapy with a prolonged course of Itraconazole (400 mg/day)
  62. 62. Bronchodilators • Airway reactivity, presumably due to transmural inflammation, is often present in patients with bronchiectasis • Aerosol bronchodilator therapy, may be appropriate but has not been studied in patients with bronchiectasis
  63. 63. Anti-inflammatory drugs Macrolide antibiotics Inhaled corticosteroids
  64. 64. Surgery Indications: Removal of focal bronchiectasis with destroyed lung ~ Removal of destroyed lung partially obstructed by a tumor or the residue of a foreign body
 ~ Reduction in overwhelming purulent and viscid sputum production
  ~ Elimination of bronchiectatic airways subject to uncontrolled hemorrhage
   ~ Removal of an area suspected of harboring resistant organisms such as MAC.